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Hypocomplementemic Urticarial Vasculitis Syndrome
Hypocomplementemic urticarial vasculitis syndrome, as opposed to urticarial vasculitis or urticarial vasculitis syndrome, is a rare disease process where the exact pathophysiology remains unknown. This article discusses the case of a 34-year-old Hispanic man with an ongoing history of chronic urticaria comprising episodes induced by low ambient temperatures, emotional stress, and spontaneous occurrences. This article serves as a consolidated reference for specialists to comprehensively review the plethora of systemic manifestations that may accompany urticarial vasculitis and highlights new systemic complications reported in association with this disease which are also observed in this case. PMID:22328958
Christensen, Jim; McCarty, Morgan
Introduction Hypocomplementemic urticarial vasculitis syndrome is a rare disorder characterized by chronic urticarial vasculitis, arthralgia, arthritis, and hypocomplementemia. Previously, only six patients with concomitant hypocomplementemic urticarial vasculitis syndrome, Jaccoud�s arthropathy, and valvular heart disease have been reported. Case presentation A 30-year-old Korean man presented with hypocomplementemic urticarial vasculitis syndrome. In addition to urticarial cutaneous lesions, he experienced polyarthralgia and arthritis that resulted in progressive deformity of the joints of both hands, cardiac valvulopathy with mitral, tricuspid, and aortic regurgitation, and intermittent neck swelling with laryngeal edema. He also developed nephritis with azotemia. His renal biopsy results revealed membranoproliferative glomerulonephritis, type I. He showed a partial response to a combination therapy of steroid, cyclophosphamide, and mycophenolate mofetil. Conclusions We describe, to the best of our knowledge, the first case of glomerulonephritis presenting a arthropathy and cardiac valvulopathy in hypocomplementemic urticarial vasculitis syndrome. A combination of corticosteroids, cyclophosphamide, and mycophenolate mofetil appear to be a safe and effective treatment for nephropathy, however are less effective for cutaneous vasculitis, cardiac valvulopathy, and arthropathy. PMID:25339233
Angioedema in a 47-year-old woman with hypocomplementemic urticarial vasculitis syndrome.
Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a type III hypersensitivity reaction characterized by urticaria with persistent acquired hypocomplementemia. Although HUVS is uncommon, it is important for physicians to be familiar with this disease, as the initial presentation is often life-threatening angioedema. The author reports the case of a 47-year-old white woman with a history of HUVS. She presented to an outpatient clinic complaining of a rash and difficulty swallowing. Urticaria and angioedema were diagnosed. The patient was given epinephrine in the clinic and then transferred to the hospital. Laboratory testing confirmed urticaria, and the patient was given intravenous methylprednisolone sodium succinate and an additional dose of epinephrine. After 1 week, the patient's angioedema improved. PMID:22331803
Jones, Julie M; Reich, Keith A; Raval, Deena G
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Urticarial Reactions: Vascular Erythema, Urticaria, Vasculitis
Vascular erythemas, including urticaria and vasculitis, represent diagnostic and therapeutic challenges. A careful systemic approach to history and physical examination should be followed by appropriate investigations to rule out systemic disease. Chronic urticaria patients should be physically tested to identify cholinergic, dermagraphic, and cold-induced responses. Food diaries and careful drug history may be important to identify exacerbating factors in chronic urticaria. A skin biopsy is necessary to diagnose vasculitis. Therapy of any underlying cause is supplemented with H1 antihistamines in urticaria patients, while vasculitis requires a different anti-inflammatory approach. PMID:21263956
Sibbald, R. Gary
BACKGROUND It has been demonstrated that neutrophils, eosinophils and monocytes, under appropriated stimulus, may express tissue factor and therefore, activate the extrinsic pathway of coagulation. We performed a transversal and case-control study of patients with chronic urticaria and patients with psoriasis, in our outpatient clinic to evaluate the production of D-dimer. OBJECTIVE To evaluate D-dimer serum levels in patients with chronic urticaria and its possible correlation with disease activity. PATIENTS AND METHODS The study was conducted from October 2010 until March 2011. We selected 37 consecutive patients from our Allergy Unit and Psoriasis Unit, and divided them into three groups for statistical analysis: (i) 12 patients with active chronic urticaria (CU); (ii) 10 patients with chronic urticaria under remission and (iii) 15 patients with psoriasis (a disease with skin inflammatory infiltrate constituted by neutrophils, lymphocytes and monocytes). Another five patients with urticarial vasculitis were allocated in our study, but not included in statistical analysis. The serum levels of D-dimer were measured by Enzyme Linked Fluorescent Assay (ELFA), and the result units were given in ng/ml FEU. RESULTS Patients with active chronic urticaria had the highest serum levels of D-dimer (p<0.01), when compared to patients with CU under remission and the control group (patients with psoriasis). CONCLUSIONS Patients with active chronic urticaria have higher serum levels of D-dimer, when compared to patients with chronic urticaria under remission and patients with psoriasis. We found elevated serum levels of D-dimer among patients with urticarial vasculitis. PMID:23793207
Criado, Paulo Ricardo; Antinori, Lidi Che Leon; Maruta, Celina Wakisaka; dos Reis, Vitor Manoel Silva
... Long-term treatment with medicines often can control the signs and symptoms of chronic vasculitis. Rarely, vasculitis doesn't respond well to treatment. This can lead to disability and even death. Much is still unknown about ...
Systemic vasculitis is one of the most challenging diseases for physicians from a diagnostic perspective. Clinical symptoms\\u000a vary according to blood vessel size and organ systems involved. This article outlines manifestations, diagnosis, and management\\u000a of the vasculitides.
Anita Krishnamurthy; Gurtej S. Cheema; M. Eric Gershwin
Hypersensitivity Vasculitis joseph July 18, 2012 No Comments What is Hypersensitivity vasculitis? Hypersensitivity vasculitis (HV) is often used to ... blood vessels, called a leukocytoclastic vasculitis. What causes Hypersensitivity vasculitis? HV may be caused by a specific ...
Synopsis Childhood vasculitis is a challenging and complex group of conditions that are multisystem in nature and often require integrated care from multiple subspecialties including rheumatology, dermatology, cardiology, nephrology, neurology, and gastroenterology. Vasculitis is defined as the presence of inflammation in the blood vessel wall. The site of vessel involvement, size of the affected vessels, extent of vascular injury, and underlying pathology determine the disease phenotype and severity. This review explores the classification and general features of pediatric vasculitis as well as the clinical presentation, diagnostic evaluation, and therapeutic options for the most common vasculitides. PMID:22560577
Weiss, Pamela F.
[Vasculitis with cutaneous necrosis induced by oral contraceptive].
Multiple ulcerated and necrotic lesions developed in a 29-year-old woman. Response to treatment during the next few months was minimal. The most plausible explanation was drug-induced vasculitis caused by contraceptive pills (Microgynon: levonorgestrel 0.15 mg and ethinyl oestradiol 0.03 mg). The diagnosis of vasculitis was confirmed by histopathological and immunofluorescent studies. Urticarial manifestations had developed into necrotizing inflammation and the lesions only began to heal after discontinuation of the pills. Contraceptive pills are not included among the drugs causing necrotizing vasculitis, and to the best of our knowledge, this is the first report. Although contraceptive pills are commonly implicated in the induction of erythema nodusom or vasculitis, such a severe reaction with necrosis is rare. PMID:1885102
Mosovich, B; Biton, A; Avinoach, I
... Drive Life As A Zebra Fundraiser 7th Annual Western Mass Golf Open Andrea Pittini � Life, Vasculitis Awareness ... Day on Saturday, October 25, 2014 at Toronto Western Hospital, Toronto, Ontario, Canada. The one-day symposium ...
Vasculitis is a spectrum of clinicopathologic disorders defined by inflammation of arteries of veins of varying caliber with variable tissue injury. Headache may be an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. A suspicion of vasculitis based on the history, clinical examination, or laboratory studies warrants prompt evaluation and treatment to forestall progression and avert cerebral ischemia or infarction. PMID:24703534
Younger, David S
Autoimmune testicular vasculitis.
A 38-year-old man presented for evaluation of new-onset left testicular pain with swelling. Testicular ultrasound revealed multiple intraparenchymal hypoechoic areas in the inferior portion of the left testicle. The patient was treated with a radical inguinal orchiectomy. Pathologic evaluation revealed intratesticular vasculitis, with involvement of medium-sized arteries. This appears to be a rare case of isolated testicular vasculitis, in the absence of any systemic symptoms. PMID:12736037
Raj, Ganesh V; Ellington, Kenneth S; Polascik, Thomas J
Inadequate understanding of the pathogenesis and etiology of vascular inflammation continues to hinder progress in the diagnosis and treatment of pediatric vasculitis. The greatest amount of work is being done in the most common vasculitides of childhood, including Kawasaki disease and Henoch-Sch�nlein purpura. Discussion of rarer types of vasculitis, on the other hand, such as antineutrophil cytoplasmic antibody-positive small vessel diseases, is largely restricted to case reports. Most aspects of the care of children with Wegener granulomatosis and microscopic polyangiitis are derived by extrapolating from data about adults. Virtually no data are available concerning ways in which these diseases may be different in children. PMID:11604599
Yalcindag, A; Sundel, R
[Symptomatic or secondary allergic vasculitis. Pathogenesis of cutaneous vasculitis].
The histopathologic alterations of the allergic cutaneous vasculitis (fibrinoid necrosis of the walls of the small blood vessels with infiltration of neuthrophils in Karyorrexis) is seen in several entities. It is possible to divide such entities in two groups: a) Primitive allergic vasculitis in which the vascular lesion is dominant and. b) Secondary allergic vasculitis in which the vascular alterations, not constant, are integrants of variable histopathologic conditions. Many classifications have deen done on clinical and pathological bases. Such classifications are not satisfactory. A classification in base of physiopathologic and immunologic mechanisms could permit adequate therapeutic planning. In this paper we consider the secondary vasculitis only. The secondary angitis are seen in: 1) Difuse colagen diseases. 3) Conditions related to infections or inflamatory processes. 3) Angitis produced by drugs. 4) Conditions produced by insects or parasites bites. 5) Heterogeneous group that include diferent tipes of not necrotizing sistemic angitis in uhich, some times, trombosant and necrotizing processes are associated. PMID:1240560
Corrales Padilla, H
Discontinuation of therapies in vasculitis.
For most patients with vasculitis, treatment will result in prevention of mortality and also lead to clinical remission. This increased survival is of course most welcome, but the burden of surviving an episode of acute vasculitis consists of the effects of the disease as well as the adverse events from treatment. Therefore, we have begun to explore the possibility of withdrawing treatment in order to avoid long-term medication toxicities. Whilst this will reduce short-term side effects, if withdrawal leads to subsequent uncontrolled flares of disease, the need for additional therapy may outweigh any benefit from a drug-free holiday. For very mild forms of vasculitis, such as isolated skin vasculitis, the best option may be to avoid treatment altogether. In those patients with vasculitis secondary to an identifiable agent such as drug toxicity or an infectious organism, discontinuing the offending drug or treating the infection will usually resolve or cure the vasculitis. In patients with localised vasculitis, surgical removal of the affected area can be curative. Other forms of vasculitis have a self-limited duration, after which there does not appear to be any clinical evidence of disease, such as is the case for the majority of patients with giant cell arteritis. By contrast, in many forms of vasculitis, especially those associated with the presence of anti-neutrophil cytoplasm antibody (ANCA), relapse occurs in at least half the patients. Where glucocorticoid therapy is used for any length of time, in doses of >5 mg/day, side effects are almost universal. Adding a concomitant agent in the attempt to shorten the course and/or reduce the dose of glucocorticoid treatment may be effective, but can also result in toxicity from the alternative agent, and leaves the patient on immunosuppressive therapy. More toxic therapy, such as cyclophosphamide, usually is administered only for a limited time or cumulative amount, in order to achieve induction of remission or flare in severe disease. The advent of targeted biologic therapy offers the opportunity to provide more effective, less toxic and perhaps more long-lasting control of disease. Rituximab in small-vessel vasculitis can result in long-lasting control of disease, for 18 months or more, from a single course of treatment. Suppression of the interleukin-6 pathway may be effective in large-vessel vasculitis. Unfortunately, none of these therapies is capable of 'cure' for the majority of patients. Therefore, discontinuation of therapy remains unachievable for most patients with vasculitis, at least in the first few years of disease. Short courses of intensive, aggressive therapy are followed by the use of maintenance treatment. Long-term follow-up studies are required to determine the potential benefit of early, more effective control of vasculitis. PMID:24129146
Luqmani, Raashid A
Cerebral vasculitis associated with cocaine abuse
A case of cerebral vasculitis in a previously healthy 22-year-old man with a history of cocaine abuse is described. Cerebral angiograms showed evidence of vasculitis. A search for possible causes other than cocaine produced no results. The authors include cocaine with methamphetamines, heroin, and ephedrine as illicit drugs that can cause cerebral vasculitis.
Kaye, B.R.; Fainstat, M.
Cerebral vasculitis in relapsing polychondritis.
Recurrent inflammation of cartilage in multiple sites is a hallmark of relapsing polychondritis (RP). Neurologic complications of this disease have begun to attract increasing attention, but the neuropathologic basis of these complications has not been described. We report a patient with RP whose autopsy showed extensive cerebral and systemic vasculitis. PMID:3336448
Stewart, S S; Ashizawa, T; Dudley, A W; Goldberg, J W; Lidsky, M D
Medium-size-artery vasculitides do occur in childhood and manifest, in the main, as polyarteritis nodosa (PAN), cutaneous PAN and Kawasaki disease. Of these, PAN is the most serious, with high morbidity and not inconsequential mortality rates. New classification criteria for PAN have been validated that will have value in epidemiological studies and clinical trials. Renal involvement is common and recent therapeutic advances may result in improved treatment options. Cutaneous PAN is a milder disease characterised by periodic exacerbations and often associated with streptococcal infection. There is controversy as to whether this is a separate entity or part of the systemic PAN spectrum. Kawasaki disease is an acute self-limiting systemic vasculitis, the second commonest vasculitis in childhood and the commonest cause of childhood-acquired heart disease. Renal manifestations occur and include tubulointerstitial nephritis and renal failure. An infectious trigger and a genetic predisposition seem likely. Intravenous immunoglobulin (IV-Ig) and aspirin are effective therapeutically, but in resistant cases, either steroid or infliximab have a role. Greater understanding of the pathogenetic mechanisms involved in these three types of vasculitis and better long-term follow-up data will lead to improved therapy and prediction of prognosis. PMID:19946711
Eleftheriou, Despina; Brogan, Paul A.
Silica exposure and systemic vasculitis.
Work in Department of Energy (DOE) facilities has exposed workers to multiple toxic agents leading to acute and chronic diseases. Many exposures were common to numerous work sites. Exposure to crystalline silica was primarily restricted to a few facilities. I present the case of a 63-year-old male who worked in DOE facilities for 30 years as a weapons testing technician. In addition to silica, other workplace exposures included beryllium, various solvents and heavy metals, depleted uranium, and ionizing radiation. In 1989 a painful macular skin lesion was biopsied and diagnosed as leukocytoclastic vasculitis. By 1992 he developed gross hematuria and dyspnea. Blood laboratory results revealed a serum creatinine concentration of 2.1 mg/dL, ethrythrocyte sedimentation rate of 61 mm/hr, negative cANCA (antineutrophil cytoplasmic antibody cytoplasmic pattern), positive pANCA (ANCA perinuclear pattern), and antiglomerular basement membrane negative. Renal biopsy showed proliferative (crescentric) and necrotizing glomerulonephritis. The patient's diagnoses included microscopic polyangiitis, systemic necrotizing vasculitis, leukocytoclastic vasculitis, and glomerulonephritis. Environmental triggers are thought to play a role in the development of an idiopathic expression of systemic autoimmune disease. Crystalline silica exposure has been linked to rheumatoid arthritis, scleroderma, systemic lupus erythematosus, rapidly progressive glomerulonephritis and some of the small vessel vasculitides. DOE workers are currently able to apply for compensation under the federal Energy Employees Occupational Illness Compensation Program (EEOICP). However, the only diseases covered by EEOICP are cancers related to radiation exposure, chronic beryllium disease, and chronic silicosis. PMID:14644669
Mulloy, Karen B
Chlamydophila pneumoniae Infection Induced Nodular Vasculitis
We present the case history of a 48-year-old male patient with Chlamydophila (Chlamydia) pneumoniae who developed a nodular vasculitis. He developed a cutaneous vasculitis with the onset of respiratory symptoms. The diagnosis of Chlamydophila pneumoniae infection was based on serology. Since this infection is very common in our population, although often asymptomatic, it should be systematically considered as a causative agent of nodular vasculitis. PMID:22220148
Sakuma, H.; Niiyama, S.; Amoh, Y.; Katsuoka, K.
Gallbladder vasculitis associated with type-1 cryoglobulinemia.
A patient with type I cryoglobulinemia and monoclonal gammopathy of uncertain significance was found to have acute gallbladder vasculitis. The most prominent manifestation was upper abdominal pain in the setting of normal liver tests. An abdominal ultrasound demonstrated a thickened gallbladder wall, along with gallstones. HIDA scanning showed a nonfunctioning gallbladder with an edematous and thickened wall. There was characteristic leukocytoclastic vasculitis affecting the gallbladder. The patient recovered uneventfully subsequent to cholecystectomy. Gallbladder vasculitis should be considered in patients with unexplained upper abdominal pain and systemic vasculitis. PMID:11281178
Rajvanshi, P; Atac, B S; Seno, R; Gupta, S
Educational Needs of Patients With Systemic Vasculitis
Behcet's Disease; Churg-Strauss Syndrome; Vasculitis, Central Nervous System; Giant Cell Arteritis; Wegener Granulomatosis; Henoch-Schoenlein Purpura; Microscopic Polyangiitis; Polyarteritis Nodosa; Takayasu's Arteritis
The mite antigen-induced histamine release from leucocytes of chronic urticarial patients was enhanced in the presence of deuterium oxide, which stabilizes microtubules. This enhancing effect of deuterium oxide on the histamine release from leucocytes may provide a useful means for the detection of allergens in vitro in chronic urticaria.
Numata, T.; Yamamoto, S.; Yamura, T.
The mite antigen-induced histamine release from leucocytes of chronic urticarial patients was enhanced in the presence of deuterium oxide, which stabilizes microtubules. This enhancing effect of deuterium oxide on the histamine release from leucocytes may provide a useful means for the detection of allergens in vitro in chronic urticaria. PMID:6168220
Numata, T; Yamamoto, S; Yamura, T
Recent aspects of vasculitis and future direction.
Vasculitis is pathologically identified as specific cellular inflammation, vessel destruction, and tissue necrosis. Current classifications of vasculitis such as the Chapel Hill Classification (CHCC) and American College of Rheumatology (ACR) guidelines are not sufficiently adequate for clinicians to diagnose vasculitis. The biomarkers that are currently in clinical use such as PR3-ANCA and MPO-ANCA, only help in diagnosing small vessel vasculitis and their sensitivity and specificity are not sufficient. However, recent developments related to the pathogenesis and etiopathogenesis of vasculitis have the potential to contribute to new and improved biomarkers. The determination of diverse roles of ANCA and synergistic effects of infection, genetic, environmental factors and drugs on pathogenesis is quite important. The demonstration of a new autoantibody directed to hLAMP-2 and the resemblance to some microbial structures, in addition to the determination of the possible roles of hepatitis B and C on vasculitis are important findings. These hints may lead to new biomarker developments, providing a better method to diagnose vasculitis. The evidence on T cell immunity as circulatory and lesional will likely contribute to the development of new drugs for vasculitis. PMID:21921363
Aras, G�lfidan
Prolastin is a commercially available form of alpha-1-antitrypsin (AAT) that is derived from pooled human plasma and used for treatment of severe alpha-1-antitrypsin deficiency (AATD). We describe a patient with AATD who developed presumed hypersensitivity vasculitis (HV) following a Prolastin infusion. Hypersensitivity vasculitis (HV), or cutaneous vasculitis, is characterized by inflammation of the small vessels of the skin with resultant ischemia to the distally supplied areas. To our knowledge, this is the first reported case of presumed hypersensitivity vasculitis following Prolastin infusion. PMID:20204065
Mwirigi, Nicola W.; Thomas, Charles F.
Prolastin is a commercially available form of alpha-1-antitrypsin (AAT) that is derived from pooled human plasma and used for treatment of severe alpha-1-antitrypsin deficiency (AATD). We describe a patient with AATD who developed presumed hypersensitivity vasculitis (HV) following a Prolastin infusion. Hypersensitivity vasculitis (HV), or cutaneous vasculitis, is characterized by inflammation of the small vessels of the skin with resultant ischemia to the distally supplied areas. To our knowledge, this is the first reported case of presumed hypersensitivity vasculitis following Prolastin infusion. PMID:20204065
Mwirigi, Nicola W; Thomas, Charles F
[Vasculitis--interdisciplinary diagnosis: radiology].
Determination of disease extension and disease activity are in the foreground of diagnostic imaging in vasculitides. There are several radiologic modalities available each having specific indications. Magnetic resonance imaging (MRI) readily depicts granulomas and mucosal inflammations in the paranasal sinuses, nasal cavity and orbits. Computed tomography detects osseous lesions of the skull. Due to its superb sensitivity MRI is an established screening modality for CNS vasculitides, although there are limitations with regard to specificity. In spite of its limited accuracy in most institutions angiography is still required for radiological confirmation of CNS vasculitis. Perfusion and diffusion MR-imaging may combine the advantages of 'conventional' MRI and angiography. By now the method is not fully validated for vasculitides, however. Vascular disease in Takayasu's arteritis and in giant cell arteritis involving predominantly large and medium sized vessels is readily diagnosed by non invasive magnetic resonance angiography. Percutaneous transluminal angioplasty has proven to be an effective and save therapeutic modality for the cure of vascular stenoses and occlusions. Plain film radiography in two planes is the established modality for pulmonary imaging. In pulmonary vasculitides a more thorough analysis of lung disease is provided by high resolution computed tomography. Diagnostic imaging does substantially assist in the interdisciplinary management of patients suffering from vasculitides. PMID:11584721
Reuter, M; Biederer, J; M�ller-H�lsbeck, S; Heller, M
Prognosis and ICU outcome of systemic vasculitis
Background Systemic vasculitis may cause life threatening complications requiring admission to an intensive care unit (ICU). The aim of this study was to evaluate outcomes of systemic vasculitis patients admitted to the ICU and to identify prognosis factors. Methods During a ten-year period, records of 31 adult patients with systemic vasculitis admitted to ICUs (median age: 63 y.o, sex ratio M/F: 21/10, SAPS II: 40) were reviewed including clinical and biological parameters, use of mechanical ventilation, catecholamine or/and dialysis support. Mortality was assessed and data were analyzed to identify predictive factors of outcome. Results Causes of ICU admissions were active manifestation of vasculitis (n?=?19), septic shock (n?=?8) and miscellaneous (n?=?4). Sixteen patients (52%) died in ICU. By univariate analysis, mortality was associated with higher SOFA (p?=?0.006) and SAPS II (p?=?0.004) scores. The need for a catecholamine support or/and a renal replacement therapy, and the occurrence of an ARDS significantly worsen the prognosis. By multivariate analysis, only SAPS II (Odd ratio: 1.16, 95% CI [1.01; 1.33]) and BVAS scores (Odd ratio: 1.16, 95% CI?=?[1.01; 1.34]) were predictive of mortality. Conclusion The mortality rate of severe vasculitis requiring an admission to ICU was high. High levels of SAPS II and BVAS scores at admission were predictive of mortality. PMID:24083831
Reproductive Health in Men and Women With Vasculitis
Giant Cell Arteritis; Takayasu's Arteritis; Polyarteritis Nodosa; Wegener's Granulomatosis; Microscopic Polyangiitis; Churg-Strauss Syndrome; Behcet's Disease; Kawasaki Disease; Henoch-schoenlein Purpura; Vasculitis, Central Nervous System; Drug-induced Necrotizing Vasculitis
Antineutrophil Cytoplasmic Antibodies, Autoimmune Neutropenia, and Vasculitis
Objectives Reports of an association between antineutrophil cytoplasmic antibodies (ANCA) and autoimmune neutropenia have rarely included cases of proven vasculitis. A case of ANCA-associated vasculitis (AAV) with recurrent neutropenia is described and relevant literature on the association between ANCA, neutropenia, and vasculitis is reviewed. Methods Longitudinal clinical assessments and laboratory findings are described in a patient with AAV and recurrent episodes of profound neutropenia from December 2008 � October 2010. A PubMed database search of the medical literature was performed for papers published from 1960 through October 2010 to identify all reported cases of ANCA and neutropenia. Results A 49 year-old man developed recurrent neutropenia, periodic fevers, arthritis, biopsy-proven cutaneous vasculitis, sensorineural hearing loss, epididymitis, and positive tests for ANCA with specificity for antibodies to both proteinase 3 and myeloperoxidase. Antineutrophil membrane antibodies were detected during an acute neutropenic phase and were not detectable in a post-recovery sample, whereas ANCA titers did not seem to correlate with neutropenia. An association between ANCA and neutropenia has been reported in 74 cases from 24 studies in the context of drug/toxin exposure, underlying autoimmune disease, or chronic neutropenia without underlying autoimmune disease. In these cases, the presence of atypical ANCA patterns and other antibodies were common; however, vasculitis was uncommon and when it occurred was usually limited to the skin and in cases of underlying toxin exposure. Conclusions ANCA is associated with autoimmune neutropenia, but systemic vasculitis rarely occurs in association with ANCA and neutropenia. The interaction between neutrophils and ANCA may provide insight into understanding both autoimmune neutropenia and AAV. PMID:21507463
Grayson, Peter C.; Sloan, J. Mark; Niles, John L.; Monach, Paul A.; Merkel, Peter A.
Pathogenesis of ANCA-Associated Vasculitis
Antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) are a group of systemic vasculitis characterized by inflammation and necrosis of blood vessel walls. Genetic, epigenetic and environmental factors contribute to the etiology and pathogenesis of AAV. Based on currently available clinical and experimental evidence, it is reasonable to conceptualize that in predisposed patients, different triggers can lead to the production of autoantibodies (ANCA) that in the context of an inflammatory environment can cause tissue inflammation and vascular injury. Several different pathways and mechanisms in the pathogenesis of AAV are described in this contemporary review. PMID:20688244
Cartin-Ceba, Rodrigo; Peikert, Tobias; Specks, Ulrich
Propylthiouracil induced leukocytoclastic vasculitis: A rare manifestation
Propylthiouracil (PTU) is a common drug used in patients with hyperthyroidism. It may cause perinuclearantineutrophil cytoplasmic antibodies (p-ANCA) in few patients with Graves� disease. This antibody has been associated with different forms of vasculitis. We report a patient who presented with cutaneous manifestations of leukocytoclasticvasculitis with simultaneous development of p-ANCAs during PTU therapy for Graves� disease. PMID:23776917
Ayturk, Semra; Demir, Mustafa Volkan; Yaylac?, Selcuk; Tamer, Ali
The impact of vasculitis on patients' social participation and friendships
Objectives Our objective is to explore how vasculitis, affects patients� friendships and social participation. Methods Vasculitis patients (n=221) completed an online questionnaire that asked if, and how, relationships with friends have changed since receiving a vasculitis diagnosis. Participants� written responses were imported into Atlas.ti, and two independent researchers used both structured and unstructured coding to identify themes. After reaching 100% consensus on the themes present in each participant�s responses, the coders determined how themes were interrelated across participants. Results Over half of patients (52%) expressed that vasculitis negatively impacted their friendships and 25% noted a negative impact on their social participation. At limes, this negative impact was related to structural changes in patients� social networks due to loss of friendships. Reduced social participation was also associated with friends� inability to understand vasculitis and its effects, vasculitis-related fatigue, and lifestyle changes such as not being able to drink alcohol and avoiding infection-prone events. Additionally, patients withdrew from social engagements due to fatigue or because of physical symptoms and side effects. Conclusion The unique circumstances associated with a rare chronic illness like vasculitis can create significant barriers to friendships, including loss of these relationships. Interventions designed to help patients cope with the social impact of vasculitis are implicated, especially if they increase patients� ability to engage in dialogue about their illness with their friends. PMID:22325346
Carpenter, Delesha M.; Meador, Amy E.; Elstad, Emily A.; Hogan, Susan L.; DeVellis, Robert F.
Idiopathic granulomatous vasculitis: response to immunosuppressive therapy.
A case of idiopathic granulomatous vasculitis (disseminated visceral giant cell arteritis) is described in an old woman, the seventh case of this rare disorder reported to date. The main organ affected was the liver and, to our knowledge, this is the first patient to be diagnosed while still alive and the only case to have received medical treatment. It is also the first time that muscular involvement has been documented in this condition. Cyclophosphamide treatment resulted in disappearance of symptoms and increase in weight. The patient died of an unrelated condition. Images PMID:7665707
Alguacil-Garcia, G F; Moreno-Requena, J; Martinez-Albadalejo, M; Hallal-Hachem, H; Gonzalez-Pina, B; de Paco-Moya, M
Leukocytoclastic vasculitis caused by drug additives.
Chronic cutaneous small vessel (leukocytoclastic) vasculitis (LCV) is a process believed to be related to the presence of circulating immune complexes. The most frequent causes and associated disorders are medications, infections, collagen vascular disorders, paraproteinemias, and, rarely, neoplasia. Reports of food or food additives as a causative factor for LCV have appeared but are rare. We report a patient with chronic cutaneous LCV in whom the presumed cause was an excipient (a dye) used in the capsule form of lithium carbonate. Furthermore, ingestion of foods containing dyes results in a disease flare in our patient. PMID:8169261
Lowry, M D; Hudson, C F; Callen, J P
Cryoglobulinemic vasculitis in a patient with CREST syndrome.
Cryoglobulinemic vasculitis is a rare entity. Although it has been reported in diffuse systemic sclerosis, it has not been reported in calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly and telangiectasia (CREST) syndrome. We report a patient with cryoglobulinemic vasculitis with CREST syndrome who did not have typical clinical features of vasculitis. This 58-year-old woman presented with mild generalized weakness and a diagnosis of CREST syndrome, which included Raynaud's syndrome, dysphagia and telangiectasias. She was positive for serum cryoglobulins, which led to a sural nerve biopsy. The biopsy results were consistent with cryoglobulinemic vasculitis. Cryoglobulinemic vasculitis has not been previously reported in CREST syndrome to our knowledge. Additionally, the patient also had limited clinical symptoms. Our patient displays the importance of checking for cryoglobulins and obtaining a nerve biopsy when the serum is positive. Both of these diagnostic tests were integral for directing appropriate treatment for this patient. PMID:24852904
Hurst, Rebecca L; Berianu, Florentina; Ginsburg, William W; Klein, Christopher J; Englestad, Janean K; Kennelly, Kathleen D
[Vasculitis : New nomenclature of the Chapel Hill consensus conference 2012].
Within the last years, many advances have been made in the understanding of the etiopathology of vasculitis as well as of different disease courses. The revised 2012 Chapel Hill consensus conference (CHCC) nomenclature reflects current knowledge on the etiopathology in addition to the descriptive principles of vessel size and types of inflammation. The anti-neutrophil cytoplasmic antibody (ANCA)-associated forms of vasculitis have been separated as a group, as opposed to immune complex small vessel vasculitis. When consensus was achieved eponyms have been replaced by systematic names, such as granulomatosis with polyangiitis (Wegener's granulomatosis) or eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). Moreover, clinically important but less well-known types of vasculitis have now been included in the CHCC nomenclature. This article presents the changes and summarizes the results of important new articles on the clinical picture and morphology of vasculitis. PMID:25269877
Holl-Ulrich, K
Cerebral large vessel vasculitis in systemic lupus erythematosus.
Neuropsychiatric systemic lupus erythematosus (NPSLE) is defined by involvement of the central nervous system in systemic lupus erythematosus (SLE), with a wide range of both neurological and psychiatric manifestations. Although its aetiopathogenesis is not fully elucidated, NPSLE seems to be a consequence of cerebral vascular pathology including thromboembolism, small-vessel vasculopathy and, in rare cases, true vasculitis. Cerebral vasculitis is rare, and cerebral large-vessel vasculitis in SLE is even more unusual. We report the case of a female patient with the diagnosis of SLE. She presented with stroke-like symptoms, headache and vertigo, and palpable purpura on her legs. Further investigations revealed that she suffered from both vasculitis of the cerebral large vessels and coexisting cutaneous small-vessel vasculitis. PMID:24969082
B�ckle, B C; Jara, D; Aichhorn, K; Junker, D; Berger, T; Ratzinger, G; Sepp, N T
Interstitial granulomatous dermatitis
interstitial granulomatous dermatitis with arthritis includes granuloma annulare, urticarial vasculitis, erythema chronicum migrans (Lyme borreliosis), rheumatoid neutrophilic dermatosis, mycosis fungoides (
Johnson, Hillary; Mengden, Stephanie; Brancaccio, Ronald R
Optic disc vasculitis in epidemic dropsy.
During an outbreak of epidemic dropsy in Delhi, 233 patients were studied. Retinal changes including venous dilatation and tortuosity, haemorrhages and disc oedema were observed. A clinical picture compatible with type I optic disc vasculitis was seen in 13 eyes and that of type II in 3 eyes. Fluorescein angiography was carried out in 23 randomly selected cases. Relevant angiographic findings included dilated and tortuous retinal veins, prominent vascular staining, blocked fluorescence, microaneurysms, disc oedema and peripapillary dye spillage. Presence of positive angiographic findings correlated well with the severity of the systemic disease, glaucoma, however, revealed no correlation. Papillophlebitis, a new ocular manifestation of Argemone mexicana oil toxicity, as also the fluorescein angiographic picture in epidemic dropsy is being reported for the first time in the literature. PMID:3430862
Sachdev, M S; Sood, N N; Mohan, M; Sachdev, H P; Gupta, S K
Livedo vasculitis associated with regional cytophagic histiocytoses.
We report a 70-year-old woman who developed recurrent multiple ulcers and residual atrophic scars on her legs and feet. The histology showed fibrinoid material in the dermal vessel walls causing partial to complete occlusion of the lumina. Capillary tufting and dilatation were also noted in the dermis. There was no vasculitis or panniculitis. Extravasated red blood cells were seen throughout the dermis; these were engulfed by benign-appearing histiocytes around the dermal-fat junction. As she was generally well and there was no evidence of the presence of cytophagic cells in her extracutaneous organs, she was diagnosed with regional phagocytic histiocytoses supposedly triggered by extravasated red blood cells. PMID:10659504
Kuno, Y; Numata, T; Tsuji, T
Vasculitis Syndromes of the Central and Peripheral Nervous Systems
... blurred vision, or blindness seizures, convulsions stroke or transient ischemic attack (TIA, sometimes also called a �mini- ... supports The Vasculitis Clinical Research Consortium (VCRC), a network of academic medical centers, patient support organizations, and ...
Rituximab induces remission in refractory HCV associated cryoglobulinaemic vasculitis
Case report: The patient was a 45 year old woman with HCV associated cryoglobulinaemic vasculitis, with purpura, arthralgia, constitutional symptoms, and a polyneuropathy. A malignant NHL was found as underlying lymphoproliferative disease. At this stage the disease was refractory to interferon ?2b and ribavirin and to subsequent immunosuppressive treatment with cyclophosphamide. Six rituximab infusions targeting the CD20 antigen on cells of the B cell lineage induced remission of the vasculitis. Bone marrow biopsy disclosed absence of the NHL. Remission has subsequently been maintained and HCV eliminated with the new pegylated interferon ?2b and ribavirin for nearly one year. Conclusions: Transition of the underlying "benign" lymphoproliferative disease to a malignant lymphoma may result in difficult to treat HCV associated cryoglobulinaemic vasculitis. Rituximab offers a new possibility for inducing remission in refractory HCV associated cryoglobulinaemic vasculitis and the lymphoproliferative disorder. After remission, HCV may subsequently be eliminated with pegylated interferon ?2b and ribavirin. PMID:14644867
Lamprecht, P; Lerin-Lozano, C; Merz, H; Dennin, R; Gause, A; Voswinkel, J; Peters, S; Gutzeit, O; Arlt, A; Solbach, W; Gross, W
Pulmonary vasculitis as the first manifestation of rheumatoid arthritis
In this report, we describe a 61-year-old man that presented with isolated pulmonary vasculitis and a positive anti-cyclic citrullinated peptide (CCP) antibody. Within a few months, the patient developed the symmetric polyarthritis consistent with rheumatoid arthritis (RA). Because the anti-CCP antibody is highly specific for RA and vasculitis is a known association of RA, we suspect the pulmonary vasculitis in this patient was the first manifestation of underlying RA. This case extends on previous reports that have shown that lung disease may predate the development of articular RA and that anti-CCP positivity and lung disease may represent a pre-RA phenotype. To our knowledge, this is the first case report of pulmonary vasculitis as the first manifestation of RA.
Tourin, Olga; de la Torre Carazo, Salvador; Smith, Daniel R.; Fischer, Aryeh
Anti-TNF-Alpha Therapy and Systemic Vasculitis
TNF-? is a pleiotropic cytokine, which plays a major role in the pathogenesis of numerous autoimmune and/or inflammatory systemic diseases. Systemic vasculitis constitutes a group of rare diseases, characterized by inflammation of the arterial or venous vessel wall, causing stenosis and thrombosis. Treatment of the different type of vasculitis mainly relies on steroids and immunosuppressive drugs. In case of refractory or relapsing diseases, however, a second line of treatment may be required. Anti-TNF-? drugs have been used in this setting during the last 15 years with inconsistent results. We reviewed herein the use of anti-TNF-? therapy in different kind of vasculitis and concluded that, except for Behcet's disease, this therapeutic option has not demonstrated significant improvement in the treatment of vasculitis. PMID:24719524
Kaplanski, Gilles
Chronic periaortitis (CP) is usually accompanied by at least mild manifestations of systemic autoimmunity; however, skin manifestations are rare. Here, we report an 82-year-old woman presenting with a pruritic annular eosinophilic dermatosis that led to the diagnosis of recurrent cutaneous eosinophilic vasculitis (RCEV) coexisting with a latent CP. The present paper is reminder that a CP should be included as a potential differential diagnosis in the elaboration of patients with cutaneous vasculitis that is suspicious of underlying autoimmunity. PMID:23198176
Kiorpelidou, Despoina; Gaitanis, Georgios; Zioga, Aikaterini; Tsili, Athina C.; Bassukas, Ioannis D.
Vasculitis is defined as inflammation of a vessel wall. Hemorrhage is defined as the escape of blood from vessels. If pulmonary vasculitis and pulmonary hemorrhage are directly connected that inflammation of blood vessels leads to rupture and then hemorrhage, the classification of the pulmonary vasculitis and pulmonary hemorrhage syndromes would be simple. There are many schemes for classifying vasculitis, such as McCluskey 1983, Jennette 1997, Vuong 2002, and Saleh 2005. Classifications can be made by etiology, histologic reaction, size and type of vessels, and associated diseases. Recently CHCC1994 is revised to CHCC 2012. Vasculitis and mimics of pulmonary vasculitis includes idiopathic necrotizing vasculitis syndromes, miscellaneous systemic disorders including collagen-vascular diseases, pulmonary hemorrhage syndromes, vascular involvement in lymphoproliferative disorders, pulmonary hypertensive arterial changes, and granulomatous vasculitis. My presentation reviews all over and focuses on pathological features of pulmonary vasculitis and pulmonary hemorrhage syndromes. PMID:25188158
Matsubara, Osamu
Complement in ANCA-Associated Vasculitis
Antineutrophil cytoplasmic autoantibodies (ANCA) are the likely cause for necrotizing small vessel vasculitis and crescentic glomerulonephritis. Unlike other forms of crescentic glomerulonephritis induced by immune complexes or anti-glomerular basement membrane (anti-GBM) antibodies that have conspicuous vessel wall immunoglobulin and complement, there is a paucity, although usually not an absence, of vessel wall immunoglobulin and complement in ANCA-associated glomerulonephritis. In spite of this comparatively lower level and more localized distribution of vessel wall complement, experimental and clinical observations strongly incriminate alternative complement pathway activation as critically important in the pathogenesis of ANCA disease. Experimental data in animal models and in vitro experiments demonstrate that primed neutrophils are activated by ANCA, which generates C5a that engages C5a receptors on neutrophils. This attracts and in turn primes more neutrophils for activation by ANCA. In patients with ANCA disease, plasma levels of C3a, C5a, soluble C5b-9 and Bb have been reported to be higher in active disease than in remission, whereas no difference was reported in plasma C4d in active versus remission ANCA disease. Thus, experimental and clinical data support the hypothesis that ANCA-induced neutrophil activation activates the alternative complement pathway and generates C5a. C5a not only recruits additional neutrophils through chemotaxis but also primes neutrophils for activation by ANCA. This creates a self-fueling inflammatory amplification loop that results in the extremely destructive necrotizing vascular injury. PMID:24161040
Jennette, J Charles; Xiao, Hong; Hu, Peiqi
CNS Vasculitis in Autoimmune Disease: MR Imaging Findings and Correlation with Angiography
BACKGROUND AND PURPOSE: MR findings in CNS vasculitis and their correlation with angiography have not been clearly defined. We therefore explored three hypotheses regarding CNS vasculitis associated with autoimmune disease: 1) MR imaging is highly sensitive; 2) a typical MR appearance exists; and, 3) MR and angiographic findings correlate well. METHODS: We studied 18 patients with CNS vasculitis associated with
Martin G. Pomper; Timothy J. Miller; John H. Stone; William C. Tidmore; David B. Hellmann
Leukocytoclastic vasculitis and acute allergic interstitial nephritis following ceftriaxone exposure
Leukocytoclastic vasculitis (LCV), also known as hypersensitivity vasculitis is a small vessel inflammatory disease which mainly involves the postcapillary venules. A 17-year-old girl developed palpable purpura over lower limbs and acute allergic interstitial nephritis 5 days after exposure to ceftriaxone. Skin biopsy from the lesion over lower limbs confirmed the diagnosis of LCV. Discontinuation of drug and treatment with steroid resulted in resolution of skin lesions and normalization of kidney functions. Beta-lactams are commonly used antibiotics in various types of infection in day-to-day practice. LCV, a rare complication of ceftriaxone should be kept in mind while using this drug.
Agrawal, Sachin R.; Rajput, Atul; Jain, A. P.
Secondary Syphilis with Nodular Vasculitis Mimicking Beh?et's Disease
Although, erythema nodosum is a common skin manifestation associated with syphilis, nodular vasculitis is a rare feature. Here, we describe a case of a 22-year-old, human immunedeficiency virus negative, non-immunocompromised man who developed recurrent oral and scrotal ulcers with nodular lesions of the lower extremitie. Beh�et's disease was initially suspected, however, his serologic test for syphilis was positive, and he was thus diagnosed with secondary syphilis, with a skin biopsy showing nodular vasculitis. The patient was treated with benzathine penicillin, and the skin lesions disappeared after treatment. PMID:24475361
Jo, Jaemin; Kim, Jae Wang; Kim, Jinseok; Yu, Jung Re
BACKGROUND/AIMS�Cryptogenetic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease whose origin is unknown. The aim of this study was to describe the clinical spectrum of CMUSE, to determine the origin and pathophysiology of the disease, and to propose a treatment strategy.?METHODS�A total of 220�French gastroenterology departments were contacted to review patients with unexplained small bowel strictures. Of 17�responses, 12�corresponded to a diagnosis of CMUSE. These patients were hospitalised between 1965�and 1993�and their medical records were reviewed.?RESULTS�All patients (mean age 42.1�(4.4) years) had intestinal and five had extraintestinal symptoms (peripheral neuropathy, buccal aphthae, sicca syndrome, polyarthralgia, Raynaud's phenomenon, arterial hypertension). One patient had heterozygous type I C2 deficiency (28�base pair gene deletion). Two to 25�(mean 8.3�(1.9)) small intestine strictures were found. Stenoses of the large jejunoileal arteries were observed on two and aneurysms on three of five mesenteric angiograms. Despite surgery, symptoms recurred in seven of 10�patients and strictures in four. Steroid therapy was effective but caused dependence. One untreated patient died. Small bowel pathology showed superficial ulceration of the mucosae and submucosae, and an inflammatory infiltrate made of neutrophils and eosinophils.?CONCLUSIONS�CMUSE is an independent entity characterised by steroid sensitive inflammation of the small bowel which often recurs after surgery. CMUSE may be related to a particular form of polyarteritis nodosa with mainly intestinal expression or with an as yet unclassified vasculitis.???Keywords: cryptogenetic multifocal ulcerous stenosing enteritis; vasculitis; small intestine PMID:11171822
Perlemuter, G; Guillevin, L; Legman, P; Weiss, L; Couturier, D; Chaussade, S
Vasculitis as the basis of cutaneous lesions in Reiter's disease
The cutaneous lesions of Reiter's disease (RD) and pustular psoriasis (PP) are said to be histologically similar and often indistinguishable. We encountered three cases of RD in which biopsy specimens of lesions clinically compatible with keratoderma blenorrhagicum showed a pustular psoriasiform tissue reaction in conjunction with a subjacent superficial leukocytoclastic vasculitis (LCV). In an attempt to ascertain if these changes
C. M Magro; A. N Crowson; R Peeling
Progress in treatment of ANCA-associated vasculitis
Autoantibodies to neutrophil cytoplasmic antigen-associated vasculitis (AAV) is characterised by inflammation of blood vessels. The introduction of immunosuppressive therapy with glucocorticoids and cyclophosphamide transformed AAV from a fatal condition to a largely treatable condition. Over the past 30 years, considerable progress has been made refining immunosuppressive regimens with a focus on minimising toxicity. There is, however, a high unmet need in the treatment of AAV. A proportion of patients are refractory to current therapies; 50% experience a relapse within 5 years and treatment toxicity contributes to mortality and chronic disability. As knowledge of the pathogenesis of vasculitis grows, it is mirrored by the availability of biological agents, which herald a revolution in the treatment of vasculitis. Lymphocyte-targeted and cytokine-targeted agents have been evaluated for the treatment of AAV and are entering the routine therapeutic arena with the potential to improve patient outcomes. As rare diseases, treatment advances in vasculitis depend on international collaborative research networks both to establish an evidence base for newer agents and to develop recommendations for patient management. PMID:22569190
Assessment of damage in vasculitis: expert ratings of damage
Objectives. Current measures of damage in vasculitis do not account for the possibility that some forms of damage may exert greater impact than others. As part of an international effort to revise how damage is quantified in vasculitis clinical research, an exercise was performed to measure expert ratings of damage items. Methods. Members of the Vasculitis Clinical Research Consortium and European Vasculitis Study Group were given a list of 129 items of damage related to WG and microscopic polyangiitis (MPA). Participants were asked to rate each item of damage on an integer scale from 0 to 10, where 10 represented the most severe form of damage and 0 indicated �no impact�. Results. A multidisciplinary panel of 50 investigators from North America, Europe and Australia�New Zealand participated. The highest median ratings (8�10) were assigned to items of damage associated with malignancy, tissue ischaemia, the central nervous system and cardiopulmonary manifestations. The mean scores ranged from 1.3 to 9.5. The highest s.d.s (?2.5) were associated with forms of damage that may benefit from surgical intervention or may not be causally associated with WG or MPA. Lower scores were assigned by nephrologists in comparison with rheumatologists and by Americans in comparison to Europeans, although the difference in median ranks used by these groups was not statistically significant (P > 0.05 for the comparisons). Conclusions. This exercise represents an important step in the development of a weighting system that may increase the utility of damage index scores for the assessment of patients with vasculitis. PMID:19454608
Jayne, David; Luqmani, Raashid; Merkel, Peter A.
Introduction Vasculitis has been associated with malignancies, more commonly hematological rather than solid malignancies. Due to the rarity of these conditions and the lack of a temporal association, the relationship between vasculitis and malignancy remains unclear. Paraneoplastic vasculitis as a phenomenon of lung cancer has been described in the literature. To the best of our knowledge, this is the first case report of leukocytoclastic vasculitis being an initial presentation of malignant pleural mesothelioma. Case presentation We report the case of an 84-year old Greek man who presented to our facility with an erythematous, pruritic and purpuric rash affecting his limbs. This was biopsy-proven to be leukocytoclastic vasculitis and treated conservatively with topical corticosteroids as well as oral prednisolone, with good results. Six months later, he was diagnosed as having malignant pleural mesothelioma. As he remained asymptomatic from his malignancy, no systemic chemotherapy was instituted. He had a recurrence of biopsy-proven leukocytoclastic vasculitis two months after he was diagnosed as having mesothelioma, which again settled with conservative measures. Conclusions It is important to remain vigilant with regard to the association between leukocytoclastic vasculitis and malignancies. A diagnosis of vasculitis requires a search for malignancies as well as other possible etiologies. This is particularly of relevance when the vasculitis becomes chronic, recurrent or treatment is no longer effective. Should our patient have experienced refractory vasculitis, we would have instituted systemic chemotherapy to treat the underlying malignancy. PMID:22937937
Sudden death due to isolated segmentary coronary vasculitis.
Coronary vasculitis is a group of conditions occurring either independently or associated with another diseases characterized by an inflammation of the blood vessel's wall and subsequent fibrinoid necrosis, occlusion, stenosis, or aneurismal dilatations. Coronary vasculitis leading to sudden cardiac death has rarely been described in the scientific literature.We present the case of an 18-year-old football player who collapsed when playing football. The patient remained in a deep coma (glasgow coma scale = 3) in the hospital for another 4 hours before dying. During hospitalization, he was diagnosed with acute anterolateral myocardial infarction with ST elevation. An autopsy was performed the next day, and on the coronary vessels were identified a dilatation with luminal extension, which, based on clinical and pathological criteria, was considered to be an isolated, coronary polyarteritis nodosa. PMID:25361059
Dermengiu, Dan; Hostiuc, Sorin; Cristian Curca, George; Constantin Rusu, Mugurel; Paparau, Cristian; Ceausu, Mihai
ANCA-associated vasculitis with renal involvement: an outcome analysis
Background. The anti-neutrophil cytoplasmic anti- body (ANCA)-associated vasculitides are a group of heterogeneous diseases. This study was undertaken to investigate the outcome of Wegener's granulomatosis (WG), microscopic polyangiitis (MPA) and renal- limited vasculitis (RLV). Furthermore, we analysed the differences in patients with proteinase 3-ANCA (PR3-ANCA) and those with myeloperoxidase-ANCA (MPO-ANCA), which have not been assessed in a homogeneously treated group
Sven Weidner; Steffen Geuss; Syrus Hafezi-Rachti; Andrea Wonka; Harald D. Rupprecht
New pathophysiological insights and treatment of ANCA-associated vasculitis
ANCA-associated-vasculitis (AAV) comprises three different diseases entities: Churg�Strauss syndrome, microscopic polyangiitis, and Wegener's granulomatosis. AAV is an autoimmune disease with complex pathophysiology. Anti-neutrophil cytoplasmic antibodies (ANCAs) with specificity for proteinase-3 (PR3) or myeloperoxidase (MPO) are hallmarks of AAV and have a pivotal role in disease development. In addition to ANCA, the cellular immune system contributes to the pathogenesis of the
Benjamin Wilde; Pieter van Paassen; Oliver Witzke; Jan Willem Cohen Tervaert
Cutaneous Small Vessel Vasculitis Accompanied by Pustulosis Palmaris et Plantaris
We present the case of a 64-year-old woman who has suffered from pustulosis palmaris et plantaris for 10 years. At the first examination, many erythematous lesions with purpura, blood crusts, and blisters were present in the lower legs and dorsum of the feet. Painful swelling in the sternal region and dorsal pain were also noted. Elevation of the CRP and myogenic enzyme levels, and liver and renal dysfunctions were noted on blood testing. Histopathologically, leukocytoclastic vasculitis was noted in small blood vessels in the whole dermal layers, and deposition of IgM and C3 in the vascular wall was detected by the direct immunofluorescence techniques. Based on these findings, cutaneous small vessel vasculitis was diagnosed. Because the patient complained of a toothache during the clinical course, an X-ray examination was performed. On pantomography, a radicular cyst and apical periodontitis were noted. The tooth symptoms changed with exacerbation and remission of the skin symptoms. These findings indicate that odontogenic infection is very likely to be a cause of cutaneous small vessel vasculitis in a manner similar to pustulosis palmaris et plantaris. PMID:22548039
Kosaka, Motoko; Kato, Tokue; Kawana, Seiji
Cytomegalovirus-related necrotising vasculitis mimicking Henoch-Sch�nlein syndrome.
Viral vasculitides have been previously reported in the literature, the role of infections in their pathogenesis ranging from direct cause to trigger event. Here we report the case of a 3-year-old immunocompetent girl who developed a systemic vasculitis leading to ileal perforation, mimicking a full blown picture of Henoch-Sch�nlein purpura. High dosage steroid treatment was started, with good response. The anatomopathological examination of the resected gastrointestinal tract showed features of necrotising vasculitis and cytomegalovirus (CMV)-related inclusion bodies in the endothelial cells, with direct correlation to vascular damage. The causative role of viral infection was revealed by the presence of CMV DNA in patient's blood and positive IgG titer against the virus. Steroid therapy was then tapered: the patient achieved clinical remission, which still persists after a six-months follow-up. Our report suggests that CMV vasculitis is probably more frequent than previously thought, even in immunocompetent patients, with a protean clinical presentation, mimicking other types of vasculitides. PMID:24854375
D'Alessandro, Matteo; Buoncompagni, Antonella; Minoia, Francesca; Coccia, Maria C; Martini, Alberto; Picco, Paolo
Primary vasculitides and vasculitis confined to skin: clinical features and new pathogenic aspects
\\u000a Abstract Cutaneous vasculitis is a heterogeneous group of disorders, and may occur with virtually all syndromes of vasculitis. It\\u000a can occur as an isolated dermatologic disorder or as a manifestation of a potentially life-threatening systemic vasculitis.\\u000a Cutaneous manifestations vary depending on the underlying cause, the size of the vessel involved and the severity and type\\u000a of inflammation. In this short
Elena Csernok; Wolfgang Ludwig Gross
Despite advances in the treatment of vasculitis, modern therapies fail to induce or maintain remission in a significant proportion of patients. Mycophenolic acid is increasingly used to treat vasculitis syndromes. Here, we consider relevant pharmacokinetic and pharmacodynamic properties of mycophenolate, with emphasis on the impact of renal impairment, and we review the existing evidence for and current trials of mycophenolate
Thomas F. Hiemstra; Rachel B. Jones; David R. W. Jayne
Kawasaki Disease as a Systemic Vasculitis in Childhood
Kawasaki disease is a disease of unknown etiology that most frequently affects infants and children under 5 years of age. Inflammation occurs in medium-sized muscular arteries throughout the body including the coronary artery, being classified as a systemic vasculitis syndrome. Histopathological investigations of Kawasaki disease have mainly focused on the coronary artery because it is directly associated with the cause of death. However, to identify the cause and pathology of Kawasaki disease, it is necessary to investigate lesions of whole organs. Thus, we attempted to review lesions in organs other than the heart and hypotheses of pathogenesis recently attracting attention. PMID:23555407
Leflunomide-induced DRESS syndrome with renal involvement and vasculitis.
DRESS or drug reaction (or rash) with eosinophilia and systemic symptoms belongs to the severe cutaneous adverse reaction group and is characterized by hematological abnormalities and visceral organ involvement. Although most often related with anticonvulsant and sulfonamide use, it is reported with numerous other drugs. We report an unusual case of DRESS syndrome due to Leflunomide, also complicated by renal involvement in the form of granulomatous interstitial nephritis and vasculitis. On a review of the literature, eight similar cases were found, and these are discussed. PMID:23271613
Pinto, Benzeeta; Dhir, Varun; Krishnan, Sabari; Nada, Ritambhra
Leukocytoclastic vasculitis presenting with acute haemorrhagic oedema of the scrotum
Fournier�s gangrene is a form of rapidly progressive necrotising fasciitis involving the soft tissues of the male genitalia. The treatment for this urologic emergency is immediate surgical debridement. Misdiagnosis of Fournier�s may lead to unnecessary surgical interventions, making careful recognition of systemic signs of illness crucial. The authors present a case of a 48-year-old patient who presented emergently with massive scrotal oedema, erythema and pain suspicious for Fournier�s gangrene and systemic signs of illness, including palpable purpura and leukocytoclastic vasculitis on biopsy. PMID:22679273
Reyner, Karina; Dahm, Philipp
Leukocytoclastic Vasculitis in a Patient with Ankylosing Spondylitis
A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later. PMID:24653851
Kobak, Senol; Yilmaz, Hatice; Karaarslan, Ahmet; Yalcin, Murat
Leukocytoclastic vasculitis in a patient with ankylosing spondylitis.
A 26-year-old male patient presented to our rheumatology clinic with pain, swelling and limitation of movement in his right ankle, and also purpuric skin lesions in the lower extremity pretibial region. He was asked questions, and he said that he had been having chronic low back pain and morning stiffness for the last few years. His physical examination revealed that he had arthritis in his right ankle, purpuric skin lesions in pretibial regions of both legs, and bilateral FABERE/FADIR positivity. The sacroiliac joint imaging and MRI revealed bilateral sacroiliitis findings, and the lateral heel imaging revealed enthesitis. HLA-B27 was positive. Skin biopsy from lower skin lesions was reported to be consistent with leukocytoclastic vasculitis. Based on clinical, laboratory, radiological, and pathological examinations, the patient was diagnosed with ankylosing spondylitis and leukocytoclastic vasculitis. Administration of corticosteroid, salazopyrin, and nonsteroid anti-inflammatory medications was started. Notable clinical and laboratory regression was observed during his checks 3 months later. PMID:24653851
Kobak, Senol; Yilmaz, Hatice; Karaarslan, Ahmet; Yalcin, Murat
T Cell-Macrophage Interactions and Granuloma Formation in Vasculitis
Granuloma formation, bringing into close proximity highly activated macrophages and T cells, is a typical event in inflammatory blood vessel diseases, and is noted in the name of several of the vasculitides. It is not known whether specific properties of the microenvironment in the blood vessel wall or the immediate surroundings of blood vessels contribute to granuloma formation and, in some cases, generation of multinucleated giant cells. Granulomas provide a specialized niche to optimize macrophage�T cell interactions, strongly activating both cell types. This is mirrored by the intensity of the systemic inflammation encountered in patients with vasculitis, often presenting with malaise, weight loss, fever, and strongly upregulated acute phase responses. As a sophisticated and highly organized structure, granulomas can serve as an ideal site to induce differentiation and maturation of T cells. The granulomas possibly seed aberrant Th1 and Th17 cells into the circulation, which are known to be the main pathogenic cells in vasculitis. Through the induction of memory T cells, aberrant innate immune responses can imprint the host immune system for decades to come and promote chronicity of the disease process. Improved understanding of T cell�macrophage interactions will redefine pathogenic models in the vasculitides and provide new avenues for immunomodulatory therapy. PMID:25309534
Hilhorst, Marc; Shirai, Tsuyoshi; Berry, Gerald; Goronzy, Jorg J.; Weyand, Cornelia M.
Gemcitabine-induced large vessel vasculitis demonstrated by PET CT: a rare, important side effect.
Gemcitabine is a nucleoside analogue used widely across haemato-oncology. Side effects are generally predictable, and typically consist of cytopenia, nausea, and infection. As the present case clearly demonstrates, gemcitabine is in rare cases associated with life-threatening large vessel vasculitis, which can involve the aorta. It is important to consider gemcitabine-induced vasculitis in non-specifically unwell patients with raised inflammatory markers and fever of unknown origin, with or without signs of vascular compromise. Early recognition, cessation of gemcitabine therapy, and high-dose steroids are critical for a good outcome. PET CT is valuable to diagnose large vessel vasculitis and monitor treatment response. PMID:24584910
Eyre, Toby A; Gooding, Sarah; Patel, Ishita; Moore, Niall; Hatton, Chris; Collins, Graham P
Issues in trial design for ANCA-associated and large-vessel vasculitis.
Randomized clinical trials (RCTs) have informed the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, although challenges still exist. The evidence base for treating large-vessel vasculitis (LVV) is weaker, but initiatives to standardize diagnostic criteria and outcome measures, and to validate biomarkers in LVV, together with newly initiated RCTs should start to address this need. In this Perspectives, we discuss the prerequisites for RCTs in vasculitis, existing trial evidence, continuing unmet needs, potential therapeutic avenues to explore and considerations in the design of future trials. PMID:24798573
Tarzi, Ruth M; Mason, Justin C; Pusey, Charles D
Advances in pathogenesis and treatment of ANCA-associated vasculitis.
Anti-neutrophil cytoplasmic autoantibodies (ANCA) directed to proteinase 3 (PR3-ANCA) and myeloperoxidase (MPO-ANCA) are sensitive and specific markers for their associated diseases, granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) and microscopic polyangiitis (MPA), respectively. Clinical observations suggest but do not prove that ANCA are involved in the pathogenesis of GPA and MPA. In vivo and in vitro experimental data strongly suggest if not prove that MPO-ANCA underlie the pathological lesions seen in MPO-ANCA associated MPA. This is less clear for PR3-ANCA associated GPA in which, besides small-vessel vasculitis, granulomatous inflammation is apparent. Here, cellular immunity appears to play an additional role. Insight into the pathogenetic events involved in these diseases has resulted in new ways of treatment that target the specific pathways that underlie the development of the lesions. PMID:25336033
Kallenberg, Cees G M
Intravitreal bevacizumab for macular edema due to occlusive vasculitis.
Systemic vasculitides can cause retinal vascular pathology, including cotton-wool spots, retinal hemorrhages, vascular occlusion, and capillary nonperfusion. Two main causes of visual decline include macular edema and retinal neovascularization. Presumably, both of these complications are caused by increased intraocular levels of vascular growth and permeability factors. We report a patient with occlusive retinal vasculitis associated with mixed connective tissue disease who was treated with intravitreal bevacizumab for chronic macular edema. One month after treatment, visual acuity improved from 20/80 to 20/60, and foveal thickness decreased from 543 microns to 306 microns. This effect persisted for at least 3 months after treatment. No complications, including increased retinal ischemia, were observed. PMID:17564933
Margolis, Ron; Lowder, Careen Y; Sears, Jonathan E; Kaiser, Peter K
Rituximab versus Cyclophosphamide for ANCA-Associated Vasculitis
BACKGROUND Cyclophosphamide and glucocorticoids have been the cornerstone of remission-induction therapy for severe antineutrophil cytoplasmic antibody (ANCA)�associated vasculitis for 40 years. Uncontrolled studies suggest that rituximab is effective and may be safer than a cyclophosphamide-based regimen. METHODS We conducted a multicenter, randomized, double-blind, double-dummy, noninferiority trial of rituximab (375 mg per square meter of body-surface area per week for 4 weeks) as compared with cyclophosphamide (2 mg per kilogram of body weight per day) for remission induction. Glucocorticoids were tapered off; the primary end point was remission of disease without the use of prednisone at 6 months. RESULTS Nine centers enrolled 197 ANCA-positive patients with either Wegener�s granulomatosis or microscopic polyangiitis. Baseline disease activity, organ involvement, and the proportion of patients with relapsing disease were similar in the two treatment groups. Sixty-three patients in the rituximab group (64%) reached the primary end point, as compared with 52 patients in the control group (53%), a result that met the criterion for noninferiority (P<0.001). The rituximab-based regimen was more efficacious than the cyclophosphamide-based regimen for inducing remission of relapsing disease; 34 of 51 patients in the rituximab group (67%) as compared with 21 of 50 patients in the control group (42%) reached the primary end point (P = 0.01). Rituximab was also as effective as cyclophosphamide in the treatment of patients with major renal disease or alveolar hemorrhage. There were no significant differences between the treatment groups with respect to rates of adverse events. CONCLUSIONS Rituximab therapy was not inferior to daily cyclophosphamide treatment for induction of remission in severe ANCA-associated vasculitis and may be superior in relapsing disease. (Funded by the National Institutes of Allergy and Infectious Diseases, Genentech, and Biogen; ClinicalTrials.gov number, NCT00104299.) PMID:20647199
Stone, John H.; Merkel, Peter A.; Spiera, Robert; Seo, Philip; Langford, Carol A.; Hoffman, Gary S.; Kallenberg, Cees G.M.; St. Clair, E. William; Turkiewicz, Anthony; Tchao, Nadia K.; Webber, Lisa; Ding, Linna; Sejismundo, Lourdes P.; Mieras, Kathleen; Weitzenkamp, David; Ikle, David; Seyfert-Margolis, Vicki; Mueller, Mark; Brunetta, Paul; Allen, Nancy B.; Fervenza, Fernando C.; Geetha, Duvuru; Keogh, Karina A.; Kissin, Eugene Y.; Monach, Paul A.; Peikert, Tobias; Stegeman, Coen; Ytterberg, Steven R.; Specks, Ulrich
This study examines the distinct gene expression profile of peripheral blood mononuclear cells from patients with chronic hepatitis C infection and mixed cryoglobulinemic (MC) vasculitis. Our DNA microarray analysis indicates that hepatitis C virus (HCV)-associated MC vasculitis is characterized by compromised neutrophil function, impaired chemotaxis, and increased interferon-stimulated gene (ISG) expression, contributing to overall MC pathogenesis and end-organ damage. Increased ISG expression is suggestive of an enhanced endogenous interferon gene signature. PBMC depletion assays demonstrate that this increased expression is likely due to an activation of monocytes and not a direct result of B cell expansion. Notably, this monocyte activation of ISG expression in HCV-associated MC vasculitis suggests a poor predictor status of interferon-based treatment. Further analysis of PBMC gene expression profiles before and after in vivo B cell depletion therapy is critical to completely understanding the mechanisms of MC vasculitis pathogenesis. PMID:24904592
Sidharthan, Sreetha; Kim, Cheol-Woo; Murphy, Alison A.; Zhang, Xiaozhen; Yang, Jun; Lempicki, Richard A.; Sneller, Michael C.; Kottilil, Shyam
Cerebral angiography as a guide for therapy in isolated central nervous system vasculitis
The authors present a case of isolated central nervous system vasculitis documented by cerebral arteriography in which remission, using a treatment regimen of prednisone and cyclophosphamide, was guided by serial arteriography during a 15-month period.
Stein, R.L.; Martino, C.R.; Weinert, D.M.; Hueftle, M.; Kammer, G.M.
Premature atherosclerosis has been observed during the course of different systemic inflammatory diseases such as rheumatoid arthritis and sytemic lupus erythematosus. Remarkably, relatively few studies have been published on the occurrence of accelerated atherosclerosis in patients with vasculitis. In giant cell arteritis (GCA), mortality because of ischaemic heart disease is not increased. In addition, intima media thickness (IMT) is lower in patients with GCA than in age-matched controls. In contrast, IMT is increased significantly in Takayasu arteritis, another form of large vessel vasculitis occurring in younger patients. In Takayasu arteritis and in Kawasaki disease, a form of medium-sized vessel vasculitis, accelerated atherosclerosis has been well documented. In small vessel vasculitis because of anti-neutrophil cytoplasmic autoantibodies-associated vasculitis, cardiovascular diseases are a major cause of mortality. IMT measurements reveal conflicting results. During active disease these patients experience acceleration of the atherosclerotic process. However, when inflammation is controlled, these patients have atherosclerotic development as in healthy subjects. Several risk factors, such as diabetes and hypertension, are present more often in patients with vasculitis compared with healthy controls. In addition, steroids may be pro-atherogenic. Most importantly, many patients have impaired renal function, persistent proteinuria and increased levels of C-reactive protein, well-known risk factors for acceleration of atherosclerosis. Enhanced oxidation processes, persistently activated T cells and reduced numbers of regulatory T cells are among the many pathophysiological factors that play a role during acceleration of atherogenesis. Finally, autoantibodies that may be relevant for acceleration of atherosclerosis are found frequently in elevated titres in patients with vasculitis. Because patients have an increased risk for cardiovascular events, vasculitis should be treated with as much care as possible. In addition, treatment should be considered with angiotensin-converting-enzyme inhibitors and/or angiotensin receptor-1 blockers, statins and acetylsalicyl acid. Finally, classical risk factors for cardiovascular disease should be monitored and treated as much as possible. PMID:19309350
Tervaert, J W Cohen
Beh�et�s disease: an immune-mediated vasculitis involving vessels of all sizes
Beh�et�s disease is an immune-mediated vasculitis affecting both small and large vessels. Small-vessel vasculitis is the pathological\\u000a basis of the multiorgan involvement that results in protean clinical features. However, relapsing aphthous ulcers in the mouth\\u000a are considered the clinical hallmark and are often also observed over the genitalia. Both manifestations, in association with\\u000a uveitis, form the typical clinical triad. In
R. Rizzi; S. Bruno; R. Dammacco
Mycophenolate mofetil (MMF) is used off-label for systemic lupus erythematosus (SLE) and systemic vasculitis. The study aim was to investigate clinical use and treatment results with MMF in patients with SLE and systemic vasculitis. This study included patients with SLE or systemic vasculitis with ongoing or previous MMF treatment. Data on treatment outcome were obtained through medical record reviews. A total of 135 of 648 (21%) patients with SLE and 43 of 455 (9%) patients with systemic vasculitis had ongoing or previous MMF treatment. Among SLE patients, the most common organ manifestation at baseline (treatment start) was renal involvement (50%). Most of the systemic vasculitis patients had Wegener's granulomatosis (GPA) (65%). Median dose of MMF was 2000?mg/day. Glucocorticoid (GC) doses were significantly reduced during MMF treatment from 21.7?mg/day at baseline to 8.3?mg/day at 12 months (p?0.05). Forty-six percent of the patients were good responders after 12 months. The most common adverse events (AES) leading to discontinuation were side effects in the gastrointestinal tract (40%) and general side effects (30%). "Survival-on-drug" analysis suggested that 40% of the patients remained on long-term MMF treatment. In conclusion, MMF was used in 21% of the SLE patients and 9% of the systemic vasculitis patients. MMF appeared to be effective with a reasonable survival-on-drug and a GC-sparing effect. PMID:24399811
Lourdudoss, C; Vollenhoven, R van
Circovirus in Tissues of Dogs with Vasculitis and Hemorrhage
We characterized the complete genome of a novel dog circovirus (DogCV) from the liver of a dog with severe hemorrhagic gastroenteritis, vasculitis, and granulomatous lymphadenitis. DogCV was detected by PCR in fecal samples from 19/168 (11.3%) dogs with diarrhea and 14/204 (6.9%) healthy dogs and in blood from 19/409 (3.3%) of dogs with thrombocytopenia and neutropenia, fever of unknown origin, or past tick bite. Co-infection with other canine pathogens was detected for 13/19 (68%) DogCV-positive dogs with diarrhea. DogCV capsid proteins from different dogs varied by up to 8%. In situ hybridization and transmission electron microscopy detected DogCV in the lymph nodes and spleens of 4 dogs with vascular compromise and histiocytic inflammation. The detection of a circovirus in tissues of dogs expands the known tropism of these viruses to a second mammalian host. Our results indicate that circovirus, alone or in co-infection with other pathogens, might contribute to illness and death in dogs. PMID:23628223
Li, Linlin; McGraw, Sabrina; Zhu, Kevin; Leutenegger, Christian M.; Marks, Stanley L.; Kubiski, Steven; Gaffney, Patricia; Dela Cruz Jr, Florante N.; Wang, Chunlin; Delwart, Eric
Current and emerging techniques for ANCA detection in vasculitis.
Detection of antineutrophil cytoplasmic antibodies (ANCAs) is a well-established diagnostic test used to evaluate suspected necrotizing vasculitis of small blood vessels. Conditions associated with these antibodies, collectively referred to as ANCA-associated vasculitides, include granulomatosis with polyangiitis (formerly known as Wegener granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome). The diagnostic utility of ANCA testing depends on the type of assay performed and on the clinical setting. Most laboratories worldwide use standard indirect immunofluorescence tests (IFT) to screen for ANCA and then confirm positive IFT results with antigen-specific tests for proteinase 3 (PR3) and myeloperoxidase (MPO). Developments such as automated image analysis of immunofluorescence patterns, so-called third-generation PR3-ANCA and MPO-ANCA ELISA, and multiplex technology have improved the detection of ANCAs. However, challenges in routine clinical practice remain, including methodological aspects of IFT performance, the diverse antigen-specific assays available, the diagnostic value of testing in clinical settings and the prognostic value of serial ANCA monitoring in the prediction of disease relapse. This Review summarizes the available data on ANCA testing, discusses the usefulness of the various ANCA assays and advises on the clinical indications for the use of ANCA testing. PMID:24890776
Csernok, Elena; Moosig, Frank
Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome.
Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. PMID:24715922
Azami, Ahad; Maleki, Nasrollah; Kalantar Hormozi, Mohammadreza; Tavosi, Zahra
Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome
Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. PMID:24715922
Azami, Ahad; Kalantar Hormozi, Mohammadreza; Tavosi, Zahra
Neuroborreliosis affects the nervous system after systemic infection with the spirochete Borrelia burgdorferi. Previously, cerebral vasculitis has been regarded as an extremely rare complication of neuroborreliosis. The data on the long-term outcome in patients with cerebral vasculitis due to neuroborreliosis are limited. The objective of this study was to perform a longitudinal analysis of cases of neuroborreliosis-associated cerebral vasculitis. We recruited all patients (n = 11) diagnosed with neuroborreliosis-associated in three neurological departments in an East German region. Inclusion criteria were sudden neurological deficits, magnetic resonance (MR) imaging findings that conform to cerebral ischemia or brain infarction, intrathecal synthesis of borrelia-specific antibodies, and non-atherosclerotic pathology of brain supplying arteries. Vasculitic changes were detected by digital subtraction angiography, MR angiography and/or transcranial Doppler ultrasound. Outcomes were measured by the modified Rankin scale (mRS) and EuroQoL Index. Cerebral vasculitis is a rare complication of Lyme disease (0.3% of all cases in the endemic area). Ten out of 11 patients diagnosed with neuroborreliosis-associated vasculitis cerebral vasculitis using clinical, radiological and immunological criteria developed ischemic stroke or transient ischemic attacks (TIA), 7 patients had recurrent stroke. Vasculitic alterations could be demonstrated in 8 patients that all except one developed ischemic lesions. The median mRS was 3 (range 0-4) at admission and 2 (range 0-6) at discharge. The posterior circulation was affected in 8 of 11 patients; thrombosis of the basilar artery was detected in 2 patients, one died in the acute stage. Neuroborreliosis can cause recurrent stroke or TIA on the basis of cerebral vasculitis. Lumbar puncture is needed for detection of this potentially life-threatening condition. Early recognition and adequate therapy would possibly improve outcome. PMID:23329377
Back, Tobias; Gr�nig, Steffi; Winter, Yaroslav; Bodechtel, Ulf; Guthke, Kersten; Khati, Diana; von Kummer, R�diger
Genome-Wide Association Study of Hepatitis C Virus- and Cryoglobulin-Related Vasculitis
Objectives The host genetic basis of mixed cryoglobulin vasculitis is not well understood and has not been studied in large cohorts. A genome-wide association study was conducted among 356 HCV RNA positive individuals with cryoglobulin-related vasculitis and 447 ethnically-matched, HCV RNA positive controls. Methods All cases had both serum cryoglobulins as well as a vasculitis syndrome. A total of 899,641 markers from the Illumina HumanOmni1-Quad chip were analyzed using logistic regression adjusted for sex, as well as genetically-determined ancestry. Replication of select single nucleotide polymorphisms (SNPs) was conducted using 91 cases and 180 controls, adjusting for sex and country of origin. Results The most significant associations were identified on chromosome 6 near the NOTCH4 and MHC class II genes. A genome-wide significant association was detected on chromosome 6 at SNP rs9461776 (OR= 2.16, p=1.16E-07) between HLA-DRB1 and DQA1: this association was further replicated in additional independent samples (meta-analysis p=7.1�10?9). Conclusions A genome-wide significant association with cryoglobulin related vasculitis was identified with SNPs near NOTCH4 and MHC Class II genes. The two regions are correlated and it is difficult to disentangle which gene is responsible for the association with MC vasculitis in this extended MHC region. PMID:25030430
Zignego, Anna Linda; Wojcik, Genevieve L.; Cacoub, Patrice; Visentini, Marcella; Casato, Milvia; Mangia, Alessandra; Latanich, Rachel; Charles, Edgar; Gragnani, Laura; Terrier, Benjamin; Piazzola, Valeria; Dustin, Lynn B.; Khakoo, Salim I.; Busch, Michael P.; Lauer, George M.; Kim, Arthur Y.; Alric, Laurent; Thomas, David L.; Duggal, Priya
Cutaneous small vessel vasculitis (CSVV) is a nonspecific finding with an extensive differential diagnosis. It is critically important to distinguish skin-limited presentations of CSVV from severe life-threatening systemic vasculitides presenting with CSVV as an initial manifestation. It can be challenging to determine which patients presenting with CSVV are at risk for systemic disease. Standard histopathologic evaluation, direct immunofluorescence, and serologic evaluation is typically required to exclude a systemic vasculitis. Type 1 cryoglobulinemia may rarely present with CSVV. Herein, we report a case of type 1 cryoglobulinemia in the setting of occult multiple myeloma. CSVV with prominent intravascular crystal formation was noted. The presence of intravascular crystals in the setting of CSVV may represent an important early clue to the diagnosis of type 1 cryoglobulinemic vasculitis. PMID:25147987
Gammon, Bryan; Longmire, Michelle; DeClerck, Brittney
Levamisole-contaminated cocaine: an emergent cause of vasculitis and skin necrosis.
The prevalence of cocaine adulterated with levamisole-induced vasculitis is increasing and physicians should be aware of this unique entity. There have been many reports of cutaneous vasculitis syndrome caused by cocaine which is contaminated with levamisole. Levamisole was used as an antihelminth drug and later was rescinded from use in humans due to adverse effects. Through this paper, we will report a 39-year-old crack cocaine user who presented with purpuric rash and skin necrosis of his ear lobes. Levamisole-induced vasculitis syndrome was suspected. A urine toxicology screen was positive for cocaine, opiates, and marijuana. Blood work revealed positive titres of ANA and p-ANCA, as well as anti-cardiolipin antibody. Biopsy taken from the left ear showed focal acute inflammation, chronic inflammation with thrombus formation, and extravasated blood cells. Treatment was primarily supportive with wound care. PMID:24778656
Souied, Osama; Baydoun, Hassan; Ghandour, Zahraa; Mobarakai, Neville
Transduction of a Foreign Histocompatibility Gene into the Arterial Wall Induces Vasculitis
NASA Astrophysics Data System (ADS)
Autoimmune vasculitis represents a disease characterized by focal inflammation within arteries at multiple sites in the vasculature. Therapeutic interventions in this disease are empirical and often unsuccessful, and the mechanisms of immune injury are not well-defined. The direct transfer of recombinant genes and their expression in the arterial wall provides an opportunity to explore the pathogenesis and treatment of vascular disease. In this report, an animal model for vasculitis has been developed. Inflammation has been elicited by direct gene transfer of a foreign class I major histocompatibility complex gene, HLA-B7, to specific sites in porcine arteries. Transfer and expression of this recombinant gene was confirmed by a polymerase chain reaction and immunohistochemistry, and cytolytic T cells specific for HLA-B7 were detected. These findings demonstrate that expression of a recombinant gene in the vessel wall can induce a focal immune response and suggest that vessel damage induced by cell-mediated immune injury can initiate vasculitis.
Nabel, Elizabeth G.; Plautz, Gregory; Nabel, Gary J.
Levamisole-Contaminated Cocaine: An Emergent Cause of Vasculitis and Skin Necrosis
The prevalence of cocaine adulterated with levamisole-induced vasculitis is increasing and physicians should be aware of this unique entity. There have been many reports of cutaneous vasculitis syndrome caused by cocaine which is contaminated with levamisole. Levamisole was used as an antihelminth drug and later was rescinded from use in humans due to adverse effects. Through this paper, we will report a 39-year-old crack cocaine user who presented with purpuric rash and skin necrosis of his ear lobes. Levamisole-induced vasculitis syndrome was suspected. A urine toxicology screen was positive for cocaine, opiates, and marijuana. Blood work revealed positive titres of ANA and p-ANCA, as well as anti-cardiolipin antibody. Biopsy taken from the left ear showed focal acute inflammation, chronic inflammation with thrombus formation, and extravasated blood cells. Treatment was primarily supportive with wound care. PMID:24778656
Mobarakai, Neville
Toxoplasmosis associated with digital vasculitis and immunodeficiency--a dilemma in diagnosis.
We present a case of acquired toxoplasmosis associated with severe digital vasculitis--a previously unreported finding. The rise in Toxoplasma gondii antibody titres may have been attenuated by impaired cellular immunity, and this poses a problem for diagnosis. PMID:6483704
Ellis, M. E.; Webb, A. K.; Dunbar, E. M.
Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis
ABSTRACT BACKGROUND Anti-neutrophil cytoplasmic autoantibodies (ANCA) specific for myeloperoxidase (MPO) or proteinase 3 (PR3) are detectable in >90% of patients with ANCA-associated vasculitis (AAV). ANCA titers do not correlate well with disease activity. In vivo and in vi...
eNOS inhibits the development of autoimmune-mediated vasculitis
Objective Many different genes or mediators have been implicated in promoting the development of vasculitis, although little is known regarding the mechanisms that normally act to suppress lesion formation. eNOS (NOS3) has been shown to inhibit vascular inflammation in many different model systems, but its roles in the pathogenesis of vasculitis have not been elucidated. The aim of this study was to determine the functions of eNOS in the initiation and progression of vasculitic lesion formation. Methods Nos3 mutant MRL/MpJ-Faslpr mice were generated and comprehensively evaluated and compared to controls for the development of autoimmune disease, including vasculitic lesion formation and glomerulonephritis. Results Nos3?/? MRL/MpJ-Faslpr mice had accelerated onset and increased incidence of renal vasculitis compared to Nos3+/+ controls. In contrast, no significant differences in severity of glomerulonephritis were observed between groups. Vasculitis was also observed in eNOS deficient mice in other organs, including increased expression in the lung. Ultrastructural analyses of renal lesions revealed the presence of electron dense deposits in affected arteries, while IgG, IgA, and C3 deposition was observed in some vessels in Nos3?/? kidneys. In addition, eNOS deficient mice showed increased levels of circulating IgG-IgA immune complexes at 20 weeks of age compared to Nos3+/+ MRL/MpJ-Faslpr and Nos3?/? C57BL/6 mice. Conclusion These findings strongly indicate that eNOS serves as a negative regulator of vasculitis in MRL/MpJ-Faslpr mice, and further suggest that NO produced by this enzyme may be critical for inhibiting lesion formation and vascular damage in human vasculitic diseases. PMID:22933338
Schoeb, Trenton R.; Jarmi, Tambi; Hicks, M. John; Henke, Scott; Zarjou, Abolfazl; Suzuki, Hitoshi; Kramer, Philip; Novak, Jan; Agarwal, Anupam; Bullard, Daniel C.
Immunological and clinical follow up of hepatitis C virus associated cryoglobulinaemic vasculitis
OBJECTIVE�To study immunological markers and compare these markers with standard measures for the clinical and immunological follow up of vasculitis activity in hepatitis C virus (HCV) associated cryoglobulinaemic vasculitis (CV).?METHODS�Serial serum samples from eight patients with newly diagnosed HCV associated CV were followed during interferon ?�treatment induced remission of the CV. Vasculitis activity and disease extent were evaluated with the Birmingham vasculitis activity score (BVAS) and disease extent index (DEI). Cryoglobulinaemia, complement levels (C3c, C4, and CH50), rheumatoid factor (RF), autoantibodies such as antinuclear antibodies, soluble interleukin 2�receptor (sIL2r), soluble intercellular adhesion molecule-1 (sICAM-1), and soluble CD30 (sCD30) were determined.?RESULTS�All patients achieved either complete or partial remission of their CV during interferon ?�treatment. There was a significant reduction in vasculitis activity and disease extent (BVAS, DEI), cryoglobulinaemia, RF, sIL2r, sICAM-1, and sCD30. Complement C3c levels increased significantly during this period. Erythrocyte sedimentation rate and levels of complement C4 and CH50 did not change significantly. Both clinical measures (BVAS and DEI) correlated significantly only with C3c and sCD30.?CONCLUSIONS�Although this study was of only a small group of patients, it shows that BVAS and DEI as clinical measures and C3c and sCD30 as immunological markers may be useful in the follow up of disease activity of HCV associated CV. The data indicate that activity of the humoral (cryoglobulinaemia, RF, autoantibodies) and cellular (sIL2r, sICAM-1, sCD30) immune response and endothelial damage (sICAM-1) are found in HCV associated CV.?? PMID:11247870
Lamprecht, P; Moosig, F; Gause, A; Herlyn, K; Csernok, E; Hansen, H; Gross, W
OBJECTIVES--(a) To assess plasma fibrinolytic parameters in patients with rheumatoid arthritis (RA) and to determine whether there are differences between patients with RA alone and those with RA complicated by vasculitis. (b) To determine if patients with RA respond differently to venous occlusion compared with normal subjects and to assess whether such a response differs in patients with RA alone and those with rheumatoid vasculitis. (c) To determine the extent of vascular damage in patients with rheumatoid vasculitis and if this correlates with the levels of plasma fibrinolytic parameters. METHODS--Sixty three patients with RA (38 had RA only and 25 had evidence of rheumatoid vasculitis) were recruited. Plasma levels of tissue plasminogen activator antigen (t-PA Ag), plasminogen activator inhibitor (PAI) activity, and factor VIII von Willebrand factor (vWF) were measured before and 10 minutes after venous occlusion. RESULTS--Patients with RA, with or without rheumatoid vasculitis, had higher baseline PAI levels than control subjects. The difference was statistically significant for patients with RA alone but was not statistically significant for patients with rheumatoid vasculitis. After venous occlusion, t-PA Ag levels increased significantly in normal subjects and patients with RA alone, but not in patients with rheumatoid vasculitis. Plasma levels of vWF were significantly higher in patients with rheumatoid vasculitis than in normal subjects and those with RA alone. In patients with RA alone, baseline vWF correlated positively with t-PA Ag levels, whereas a negative correlation was found between these two parameters in patients with rheumatoid vasculitis. A negative correlation between vWF and t-PA Ag levels after venous occlusion was also found in patients with rheumatoid vasculitis. CONCLUSIONS--Patients with rheumatoid vasculitis showed evidence of vascular damage with increased levels of vWF and impaired t-PA Ag release after venous occlusion, a useful measurement of endothelial reserve to remove fibrin. This may be of pathophysiological importance in the development of vasculitis in these patients. PMID:8239758
Lau, C S; McLaren, M; Hanslip, J; Kerr, M; Belch, J J
Metastatic Pulmonary Calcinosis and Leukocytoclastic Vasculitis in a Patient with Multiple Myeloma
Both leukocytoclastic vasculitis and metastatic pulmonary calcification are conditions that rarely occur during the course of multiple myeloma. We present a multiple myeloma patient that had severe dyspnea due to metastatic pulmonary calcinosis, and ulceronecrotic skin lesions caused by leukocytoclastic vasculitis. After 3 courses of standarddose chemotherapy all skin and pulmonary lesions disappeared. Autologous peripheral stem cell transplantation was performed and during 1 year of follow-up the patient was in complete remission; after 1 year, laboratory test results indicated disease relapse. Although the patient was treated with bortezomib and dexamethasone, the disease progressed. Non-myeloablative allogeneic stem cell transplantation was performed, but despite of all treatment the patient died due to disease progression. Conflict of interest:None declared. PMID:24385728
�a??rgan, Se�kin; Soyer, Nur; Vural, Filiz; Saydam, G�ray; Y?ld?r?m ?im?ir, Ilg?n; D�nmez, Ayhan; Akal?n, Taner; Bi�ero?lu, Selen; Tombulo?lu, Murat
A case of primary systemic necrotizing vasculitis presenting primarily with neurologic involvement.
Systemic necrotizing vasculitis (SNV) is a type of vasculitis that presents with necrosis, predominantly involving large, medium-sized and small arteries. Peripheral neuropathy is a major clinical feature of the primary and secondary systemic vasculitides, and is often observed during the early phases of the disease, causing axonal neuropathy. The prevalence of central nervous system (CNS) involvement ranges from 4% to 45%. Encephalopathy, focal neurological deficits, and seizures are the most common manifestations and usually occur late during the course of SNV. In this report, we describe a 61-year-old woman with SNV who had both CNS and peripheral nervous system vasculitic involvement. We also discuss the pathophysiology of nervous system involvement in patients with SNV. PMID:24854993
Liu, Jing-Yao; Zhang, Ren-Sheng; Zhou, Chun-Kui
The systemic vasculitides are multifocal diseases characterized by the presence of blood vessel inflammation in multiple organ systems. Their clinical presentation is variable extending from self-limited illness to critical complications including diffuse alveolar hemorrhage and glomerulonephritis. Alveolar hemorrhage is a life-threatening manifestation of pulmonary vasculitis that can rapidly progress into acute respiratory failure requiring ventilatory support. We present the case of a 74-year-old patient admitted to the Intensive Care Unit with severe hypoxic respiratory failure and diffuse alveolar infiltrates in chest imaging that was later diagnosed as antineutrophil cytoplasmic antibodies-associated vasculitis. The report highlights the importance of differentiate between alveolar hemorrhage and acute respiratory distress syndrome of other etiology because alveolar hemorrhage is reversible with prompt initiation of treatment.
Sourla, Evdokia; Bagalas, Vasilis; Tsioulis, Helias; Paspala, Asimina; Akritidou, Sofia; Pataka, Athanasia; Fekete, Katalin; Kioumis, Ioannis P.; Stanopoulos, Ioannis; Pitsiou, Georgia
Levamisole-induced myopathy and leukocytoclastic vasculitis: a case report and literature review.
Levamisole, an immunomodulator and anthelmintic medication, has been used in dermatology for years. Even though the adverse effects are usually mild and reversible, attention should be paid toward severe events such as vasculitis and neutropenia. To the best of our knowledge, this is the first case report on a patient presenting with myopathy caused by levamisole. Here, we report a 34-year-old woman with recalcitrant warts who received levamisole 100?mg daily for 5?days. Subsequently, bilateral lower limb weakness accompanied by multiple painful and non-blanchable purpura was noted. Levamisole-induced myopathy and leukocytoclastic vasculitis were diagnosed by skin histopathology, direct immunofluorescence, and electromyography. After discontinuing levamisole and giving a short course of systemic steroid, these symptoms demonstrated a resolving trend. PMID:24552411
Tsai, Meng-Hsuan; Yang, Jen-Hung; Kung, Sheng-Ling; Hsiao, Yu-Ping
Silica and asbestos exposure in ANCA-associated vasculitis with pulmonary involvement.
Silica and asbestos exposure are thought to belong to the triggering factors of antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis. We carried out a study to find out whether patients with pulmonary involvement attributable to ANCA-associated vasculitis (AAV) have been exposed to silicon-containing materials. Thirty-one patients (12 women, 19 men, median age 51 years) were interviewed using a structured questionnaire. Occupational exposure to silicon-containing chemicals was reported by 22.6% of the patients (12.9% to SiO2, 9.7% to asbestos), compared with 0% of control subjects (p<0.05). Our findings support the pathophysiologic role of silica in AAV. PMID:16153001
Rihova, Zuzana; Maixnerova, Dita; Jancova, Eva; Pelclova, Daniela; Bartunkova, Jirina; Fenclova, Zdenka; Vankova, Zdenka; Reiterova, Jana; Merta, Miroslav; Rysava, Romana; Tesar, Vladimir
p-ANCA-Associated Vasculitis Caused by Levamisole-Adulterated Cocaine: A Case Report
A rare case of a patient with an unusual skin rash after using cocaine is presented. A clinical diagnosis of levamisole-induced vasculitis was made based on initial presentation of purpuric skin lesions involving the ears and positive cocaine on urine toxicology screening. The diagnosis was confirmed after laboratory findings of neutropenia, positive serum levamisole, and a histopathologic test of the skin lesions. The pathophysiology of this condition is discussed. Treatment with methylprednisolone and prednisone led to the resolution of the neutropenia and complete recovery of the skin lesions. With the growing use of levamisole-tainted cocaine, clinicians should be aware of the symptoms of vasculitis and neutropenia induced by this combination of drugs to avoid unnecessary tests and delayed diagnosis. PMID:23476825
Carter, Michelle R.
[Vasculitis 2013. What are the changes introduced in the 2012 Chapel Hill Consensus Conference?].
Vasculitis is a heterogeneous group of rare disorders in which inflammation of blood vessels is the common feature. Due to the increasing number of diseases as well as overlaps and gaps in the definition and nomenclature, the classification criteria were constantly changing in the past decades. The classifications were based essentially on the size of affected blood vessels and pathologic characteristics of inflamed vessel walls. The standard procedures and validated diagnostic criteria are missing from the diagnostics of vasculitis, thus in clinical practice the classification criteria are applicable. The 2012 Chapel Hill Consensus Conference brought a change in the definition, nomenclature and classification of previously uncategorized diseases. The definitions of subgroups accurately determine the diagnosis of the specific disease, and they are suitable for establishing homogeneous disease groups. By better understanding of the etiopathogenetic factors, further diseases and subgroups may be defined in the near future. PMID:24077160
Zeher, Margit
Blocking the NOTCH Pathway Inhibits Vascular Inflammation in Large Vessel Vasculitis
Background Giant cell arteritis (GCA) is a granulomatous vasculitis of the aorta and its branches that causes blindness, stroke and aortic aneurysm. CD4 T-cells are key pathogenic regulators, instructed by vessel wall dendritic cells (DC) to differentiate into vasculitic T cells. The unique pathways driving this DC-T-cell interaction are incompletely understood but may provide novel therapeutic targets for a disease in which the only established therapy is chronic treatment with high doses of corticosteroids. Methods and Results Immunohistochemical and gene expression analysis of GCA-affected temporal arteries revealed abundant expression of the NOTCH receptor and its ligands Jagged1 and Delta1. Cleavage of the NOTCH intracellular domain (NICD) in wall-infiltrating T cells indicated ongoing NOTCH pathway activation in large vessel vasculitis. NOTCH activation did not occur in small vessel vasculitis affecting branches of the vasa vasorum tree. We devised two strategies to block NOTCH pathway activation; ?-secretase inhibitor treatment, preventing nuclear translocation of the NICD, and competing for receptor-ligand interactions through excess soluble ligand, Jagged1-Fc. In humanized mice carrying human arteries NOTCH pathway disruption had strong immunosuppressive effects, inhibiting T-cell activation in the early and established phase of vascular inflammation. NOTCH inhibition was particularly effective in downregulating Th17 responses, but also markedly suppressed Th1 responses. Conclusions Blocking NOTCH signaling depleted T cells from the vascular infiltrates, implicating NOTCH-NOTCH ligand interactions in regulating T-cell retention and survival in vessel wall inflammation. Modulating the NOTCH signaling cascade emerges as a promising new strategy for immunosuppressive therapy of large vessel vasculitis. PMID:21220737
Piggott, Kisha; Deng, Jiusheng; Warrington, Kenneth; Younge, Brian; Kubo, Jessica T.; Desai, Manisha; Goronzy, Jorg J.; Weyand, Cornelia M.
Bioinspired engineering study of Plantae vascules for self-healing composite structures
This paper presents the first conceptual study into creating a Plantae-inspired vascular network within a fibre-reinforced polymer composite laminate, which provides an ongoing self-healing functionality without incurring a mass penalty. Through the application of a �lost-wax� technique, orthogonal hollow vascules, inspired by the �ray cell� structures found in ring porous hardwoods, were successfully introduced within a carbon fibre-reinforced epoxy polymer composite laminate. The influence on fibre architecture and mechanical behaviour of single vascules (located on the laminate centreline) when aligned parallel and transverse to the local host ply was characterized experimentally using a compression-after-impact test methodology. Ultrasonic C-scanning and high-resolution micro-CT X-ray was undertaken to identify the influence of and interaction between the internal vasculature and impact damage. The results clearly show that damage morphology is influenced by vascule orientation and that a 10 J low-velocity impact damage event is sufficient to breach the vasculature; a prerequisite for any subsequent self-healing function. The residual compressive strength after a 10 J impact was found to be dependent upon vascule orientation. In general, residual compressive strength decreased to 70 per cent of undamaged strength when vasculature was aligned parallel to the local host ply and a value of 63 per cent when aligned transverse. This bioinspired engineering study has illustrated the potential that a vasculature concept has to offer in terms of providing a self-healing function with minimum mass penalty, without initiating premature failure within a composite structure. PMID:19955122
Trask, R. S.; Bond, I. P.
Postoperative spinal infection mimicking systemic vasculitis with titanium-spinal implants
Background Secondary systemic vasculitis after posterior spinal fusion surgery is rare. It is usually related to over-reaction of immune-system, to genetic factors, toxicity, infection or metal allergies. Case Description A 14 year-old girl with a history of extended posterior spinal fusion due to idiopathic scoliosis presented to our department with diffuse erythema and nephritis (macroscopic hemuresis and proteinuria) 5 months post surgery. The surgical trauma had no signs of inflammation or infection. The blood markers ESR and CRP were increased. Skin tests were positive for nickel allergy, which is a content of titanium alloy. The patient received corticosteroids systematically (hydrocortisone 10 mg) for 6 months, leading to total recess of skin and systemic reaction. However, a palpable mass close to the surgical wound raised the suspicion of a late infection. The patient had a second surgery consisting of surgical debridement and one stage revision of posterior spinal instrumentation. Intraoperative cultures were positive to Staphylococcus aureus. Intravenous antibiotics were administered. The patient is now free of symptoms 24 months post revision surgery without any signs of recurrence of either vasculitis or infection. Literature Review Systemic vasculitis after spinal surgery is exceptionally rare. Causative factors are broad and sometimes controversial. In general, it is associated with allergy to metal ions. This is usually addressed with metal on metal total hip bearings. In spinal surgery, titanium implants are considered to be inert and only few reports have presented cases with systemic vasculitides. Therefore, other etiologies of immune over-reaction should always be considered, such as drug toxicity, infection, or genetic predisposition. Purposes and Clinical Relevance Our purpose was to highlight the difficulties during the diagnostic work-up for systemic vasculitis and management in cases of posterior spinal surgery. PMID:21914179
Surgical lung biopsy to diagnose Behcet's vasculitis with adult respiratory distress syndrome.
A 34-year-old female presented with fever and abdominal pain. Past medical history includes Crohn's and Behcet's disease. Examination revealed multiple skin ulcerations, oral aphthae, and bilateral coarse rales. She developed respiratory distress with diffuse bilateral alveolar infiltrates on chest radiograph requiring intubation. PaO2/FiO2 ratio was 132. The chest computed tomography revealed extensive nodular and patchy ground-glass opacities. Bronchoalveolar lavage demonstrated a predominance of neutrophils. Methylprednisolone 60 mg every 6 h and broad-spectrum antimicrobials were initiated. No infectious etiologies were identified. Surgical lung biopsy demonstrated diffuse alveolar damage (DAD) mixed with lymphocytic and necrotizing vasculitis with multiple small infarcts and thrombi consistent with Behcet's vasculitis. As she improved, steroids were tapered and discharged home on oral cyclophosphamide. Pulmonary involvement in Behcet's is unusual and commonly manifests as pulmonary artery aneurysms, thrombosis, infarction, and hemorrhage. Lung biopsy findings demonstrating DAD are consistent with the clinical diagnosis of adult respiratory distress syndrome. The additional findings of necrotizing vasculitis and infarcts may have led to DAD. PMID:25378849
Vydyula, Ravikanth; Allred, Charles; Huartado, Mariana; Mina, Bushra
The purpose of this study was to evaluate the safety and effectiveness of percutaneous transluminal angioplasty for occlusive arterial disease associated with vasculitis. Eleven patients(10 women, 1 man; ages 35-82 years) with the diagnosis of vasculitis of the large vessels underwent interventional treatment during intraarterial angiography. The causes included giant cell arteritis(n = 8) and Takayasu arteritis (n = 3).Thirty-three occlusive lesions (including brachiocephalic and renalarteries, and arteries of upper and lower extremities) were treated with balloon angioplasty and/or stent placement. Follow-up included clinical examination, angiography, and color duplex ultrasound.Technical success was 100% (25/25) for stenoses and 50% (4/8) for occlusive lesions, representing all lesions combined from different anatomic locations. Dissection (n = 3) and arterial rupture with retroperitoneal hematoma (n = 1) was found in three patients. During follow-up (mean 12 months), restenoses(n = 8) and re-restenoses (n = 1)occurred in 8 vascular areas. Three of these lesions were treated with repeated PTA (n = 4). The cumulative primary clinical success rate was 67.6%, cumulative secondary success rate 74.4%, and cumulative tertiary success rate 75.9%. Interventional therapy in systemic vasculitis provides promising results in technical success rates and followup. Angioplasty may result in arterial injury, but the rate of complications is low.
Both, M.; Jahnke, T. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel(Germany); Reinhold-Keller, E. [Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt (Germany); Reuter, M.; Grimm, J.; Biederer, J.; Brossmann, J. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel (Germany); Gross, W.L. [Department of Rheumatology, University of Luebeck, Rheumaklinik Bad Bramstedt (Germany); Heller, M.; Mueller-Huelsbeck, S. [Department of Radiology, Christian-Albrechts-University of Kiel, Kiel (Germany)
Vasculitis in systemic lupus erythematosus (SLE) has a broad spectrum of clinical manifestations from cutaneous to visceral involvement and its prognosis ranges from mild to life-threatening. We report the case of a previously healthy 17-year-old woman with eight months' history of arthralgias and myalgias. Subsequently, she developed facial and lower limbs edema, and hair loss. Two weeks before admission to a secondary level hospital, she developed fever up to 40?? followed by abdominal pain, rectal bleeding, hematemesis and blisters on both legs, reason for which she was hospitalized. With active bullous SLE with rapidly progressive glomerulonephritis suspected, she was treated with methylprednisolone pulses without response. After one week of treatment, she was transferred to a tertiary level hospital. On admission she presented acute arterial insufficiency of the lower extremities, respiratory failure with apnea, metabolic acidosis and shock; six hours later she died. Autopsy findings showed active diffuse lupus nephritis and diffuse systemic vasculitis that involved vessels from the skin, brain, myocardium, spleen, iliac and renal arteries. In addition, serositis of the small intestine and colon, acute and chronic pericarditis, pericardial effusion and myocarditis were found. Immunologic tests confirmed SLE diagnosis. In this case the fulminant course was the result of SLE high disease activity, visceral vasculitis of several organs and late diagnosis, referral and treatment. Early diagnosis, and opportune referral to the rheumatologist for intensive treatment can improve the outlook in these patients. PMID:25107938
Medina, G; Gonz�lez-P�rez, D; V�zquez-Ju�rez, C; S�nchez-Uribe, M; Saavedra, M A; Jara, L J
Antiproteinase 3 antibodies (antiPR3) are assumed to be subtypes of antineutrophil cytoplasmic autoantibodies (ANCA), with a high specificity for active Wegener's granulomatosis and microscopic polyangiitis. Thus, antiPR3 positivity in ELISA, together with negativity in indirect immunofluorescence (IIF) is a rare finding. A 56-year-old man with Dupuytren's contracture and polyneuropathy was admitted for leukocytoclastic vasculitis. Echocardiography, performed because of fever and dyspnea, detected aortic valve endocarditis. Because of severe aortic insufficiency the valve was replaced. Blood cultures and bacteriologic investigations of the explanted valve were negative. AntiPR3 were elevated (123-163 U/ml; normal <6 U/ml), together with negativity in IIF. This case shows that antiPR3 elevation with negative ANCA may be associated with vasculitis, endocarditis, polyneuropathy and Dupuytren's contracture. A causal relationship between the clinical presentation and antiPR3 elevation is likely. In order not to miss such cases of vasculitis, combined screening by IIF and ELISA is recommended in selected cases. PMID:12918859
St�llberger, Claudia; Finsterer, Josef; Zlabinger, Gerhard J; Weihsengruber, Felix; Redtenbacher, Susanne; Bonner, Gerhard; Herkner, Kurt; Deutsch, Manfred
Introduction. Goodpasture's syndrome is a rare clinical entity and is characterized by circulating autoantibodies which are principally directed against the glomerular/alveolar basement membrane. The etiology of Goodpasture's syndrome is still unknown. Lung involvement occurs as a result of lung injury and the exposure of new epitopes to the immune system. Recently, several studies have suggested the role of silica as one of etiological factors in ANCA associated vasculitis and glomerulonephritis. Materials and Methods. We present a case of a 40-year-old welder with silicosiderosis, who developed anti-GBM disease with p-ANCA positivity. Case Report. Patient presented to an emergency with gradually increasing breathlessness along with renal failure and died after short hospital stay. Autopsy pathology findings revealed crescentic glomerulonephritis with linear glomerular basement membrane antibody deposition, splenic vasculitis, pulmonary haemorrhage, and pulmonary silicosiderosis. Conclusion. This case reinforces the role of environmental triggers like exposure to silica, metal dust, and tobacco in pathogenesis of Goodpasture's syndrome and p-ANCA associated vasculitis.
Bal, Amanjit; Das, Ashim; Gupta, Dheeraj; Garg, Mandeep
Objective The development of pathogenic anti-neutrophil cytoplasmic autoantibodies (ANCAs) can result in systemic small vessel vasculitis. However, the breakdown in immune tolerance that results in the induction and persistence of ANCAs is not well-understood. We hypothesized that abnormal T cell regulation is central to disease pathogenesis and demonstrate here two separate abnormalities in T cell regulation in ANCA-associated vasculitis patients. Methods Peripheral blood samples were obtained from patients with ANCA-associated vasculitis (n=63) and healthy controls (n=19) for flow cytometric analysis of CD4+ T cell populations. Functional T cell studies were performed with FACS sorted CD4+ T cell populations stimulated with anti-CD3/28. Results First, we show that the Treg frequency in the peripheral blood of active disease patients is increased, but Tregs from patients with ANCA-associated vasculitis have decreased suppressive function. Tregs from active disease patients disproportionately utilize a FOXP3 isoform lacking exon 2, which may alter Treg function. Second, we identify a CD4+ T cell population with increased frequency that is resistant to Treg suppression, produces pro-inflammatory cytokines, and is antigen-experienced. Conclusion ANCA-associated vasculitis is associated with disruption of the suppressive Treg network and increased frequency of a distinct pro-inflammatory effector T cell subset which comprises the majority of peripheral CD4+ T cells. PMID:23553415
Free, Meghan E; Bunch, Donna O; McGregor, JulieAnne; Jones, Britta E; Berg, Elisabeth A; Hogan, Susan L; Hu, Yichun; Preston, Gloria A; Jennette, J. Charles; Falk, Ronald J; Su, Maureen A
Circulating Markers of Vascular Injury and Angiogenesis in ANCA-Associated Vasculitis
Objective To identify biomarkers that distinguish between active ANCA-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation. Methods Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV. 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis (RAVE) trial were studied. Serum levels of E-selectin, ICAM-3, MMP1, MMP3, MMP9, P-selectin, thrombomodulin, and VEGF were measured at study screening (time of active disease) and at month 6. ESR and CRP levels had been measured at the time of the clinical visit. The primary outcome was the difference in marker level between screening and month 6 among patients in remission (BVAS/WG score of 0) at month 6. Results All subjects had severe active vasculitis (mean BVAS/WG score 8.6 +/? 3.2 SD) at screening. Among the 123 subjects clinically in remission at month 6, levels of all markers except E-selectin showed significant declines. MMP3 levels were also higher among the 23 subjects with active disease at month 6 than among the 123 subjects in remission. MMP3 levels correlated weakly with ESR and CRP. Conclusion Many markers of vascular injury and angiogenesis are elevated in severe active AAV and decline with treatment, but MMP3 appears to distinguish active AAV from remission better than the other markers studied. Further study of MMP3 is warranted to determine its clinical utility in combination with conventional markers of inflammation and ANCA titers. PMID:21953143
Monach, Paul A; Tomasson, Gunnar; Specks, Ulrich; Stone, John H; Cuthbertson, David; Krischer, Jeffrey; Ding, Linna; Fervenza, Fernando C; Fessler, Barri J; Hoffman, Gary S; Ikle, David; Kallenberg, Cees GM; Langford, Carol A; Mueller, Mark; Seo, Philip; St.Clair, E William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R; Gu, Yi-Zhong; Snyder, Ronald D; Merkel, Peter A
A 52-year-old man presented with a severe head drop and proximal extremity weakness. Magnetic resonance imaging of the cervical spine showed T2 hyperintensity in cervical paraspinal muscles. Electrodiagnostic studies revealed an axial myopathy isolated to paraspinal muscles. A splenius capitis muscle biopsy confirmed an acute myopathy associated with nonsystemic vasculitis. The patient improved on steroids, intravenous immunoglobulin, and monthly pulse doses of cyclophosphamide. Our case emphasizes that a subgroup of patients with dropped head syndrome have treatable conditions. PMID:22538306
Nielsen, Amy Almaraz; Smith, Benn E; Engel, Andrew G; Bosch, Erich Peter
Introduction Adenocarcinoma of the seminal vesicles is a very rare malignancy, with less than 100 cases reported worldwide. It is documented to have a poor prognosis, with the majority of patients developing metastatic disease, most commonly in the prostate, bladder and rectum. Currently there is no standard treatment for metastatic disease and the limited reports of treatment with radiotherapy, chemotherapy and hormonal (anti-androgenic) therapy show that they are generally of modest benefit. The association between malignancy and an increased risk of autoimmune vasculitis has been demonstrated in a number of malignancies, but to date there have been no documented cases of adenocarcinoma of the seminal vesicles associated with anti-neutrophil cytoplasmic antibody vasculitis. Case presentation In this report we describe the case of a 55-year-old Caucasian man with metastatic adenocarcinoma of the seminal vesicles. He previously had received chemotherapy treatment for advanced testicular cancer and later presented with hemospermia. He subsequently developed c-antineutrophil cytoplasmic antibody vasculitis requiring intensive immunosuppression and renal dialysis. Conclusion Adenocarcinoma of the seminal vesicles is a rare diagnosis and our case is more unusual in that our patient previously had chemotherapy treatment for advanced testicular cancer and went on to develop severe antineutrophil cytoplasmic antibody vasculitis when diagnosed with metastatic seminal vesicle cancer. This case illustrates that autoimmune vasculitis can occur in any patient with malignancy and an early referral to the renal team combined with renal biopsy can assist in the earlier diagnosis and more successful management of these rare events. This case should be of interest to oncologists, renal physicians, urologists and general physicians who encounter patients presenting with hemospermia or vasculitis. PMID:23452402
Vasculitis of medium- and large-sized arteries is an inflammatory and stenotic disease characterized by a strong predilection for the aortic arch and its branches. It presents with symptoms and signs as per the vessels and organs involved. Pulmonary sequestration is a rare abnormality characterized by a mass of nonfunctioning lung tissue that receives its vascular supply from a systemic artery and is separated from the normal tracheobronchial tree. The following is a rare case report showing the presence of pulmonary sequestration in a patient with recently diagnosed hypertension and intestinal angina due to medium and large vessel vasculitis. PMID:24778487
Malik, Sarthak; Khurana, Sakshi; Vasudevan, Vishnu; Gupta, Nikhil
Vasculitis of medium- and large-sized arteries is an inflammatory and stenotic disease characterized by a strong predilection for the aortic arch and its branches. It presents with symptoms and signs as per the vessels and organs involved. Pulmonary sequestration is a rare abnormality characterized by a mass of nonfunctioning lung tissue that receives its vascular supply from a systemic artery and is separated from the normal tracheobronchial tree. The following is a rare case report showing the presence of pulmonary sequestration in a patient with recently diagnosed hypertension and intestinal angina due to medium and large vessel vasculitis. PMID:24778487
Malik, Sarthak; Khurana, Sakshi; Vasudevan, Vishnu; Gupta, Nikhil
Using an ELISA, anti-endothelial cell antibodies (AECA) have been found in sera obtained at the time of renal biopsy in 46 out of 57 patients (81%) with systemic lupus erythematosus (SLE) and nephritis (mean binding index (BI)=84%�52.8) compared with 22 out of 50 SLE patients (44%) without nephritis (mean BI=45%�35.9). Seventy normal human sera had a mean BI of 10%�9.8. The highest levels were seen in patients with diffuse proliferative glomerulonephritis (WHO grade IV) and in patients with proteinuria and nephrotic syndrome. When the biopsies were assessed for activity and chronicity scores, AECA were associated with active renal lesions (P<0.001). AECA levels correlated with low complement levels but not with anti-DNA antibodies to extractable nuclear antigens (ENA), anti-cardiolipin or anti-neutrophil cytoplasmic antibodies. The presence of AECA conferred a positive predictive value of 0.68 for the presence of nephritis. Twenty-five patients had active vasculitis at the time of assay and the highest AECA values were seen in patients with both nephritis and vasculitis. No correlation was seen with serum immunoglobulin levels and immune complexes did not bind significantly to the endothelial surface. The possible role of these antibodies as a marker in lupus nephritis is discussed. PMID:1864005
D'Cruz, D. P.; Houssiau, F. A.; Ramirez, G.; Baguley, E.; McCutcheon, J.; Vianna, J.; Haga, H.-J.; Swana, G. T.; Khamashta, M. A.; Taylor, J. C.; Davies, D. R.; Hughes, G. R. V.
Using an ELISA, anti-endothelial cell antibodies (AECA) have been found in sera obtained at the time of renal biopsy in 46 out of 57 patients (81%) with systemic lupus erythematosus (SLE) and nephritis (mean binding index (BI) = 84% +/- 52.8) compared with 22 out of 50 SLE patients (44%) without nephritis (mean BI = 45% +/- 35.9). Seventy normal human sera had a mean BI of 10% +/- 9.8. The highest levels were seen in patients with diffuse proliferative glomerulonephritis (WHO grade IV) and in patients with proteinuria and nephrotic syndrome. When the biopsies were assessed for activity and chronicity scores, AECA were associated with active renal lesions (P less than 0.001). AECA levels correlated with low complement levels but not with anti-DNA antibodies to extractable nuclear antigens (ENA), anti-cardiolipin or anti-neutrophil cytoplasmic antibodies. The presence of AECA conferred a positive predictive value of 0.68 for the presence of nephritis. Twenty-five patients had active vasculitis at the time of assay and the highest AECA values were seen in patients with both nephritis and vasculitis. No correlation was seen with serum immunoglobulin levels and immune complexes did not bind significantly to the endothelial surface. The possible role of these antibodies as a marker in lupus nephritis is discussed. PMID:1864005
D'Cruz, D P; Houssiau, F A; Ramirez, G; Baguley, E; McCutcheon, J; Vianna, J; Haga, H J; Swana, G T; Khamashta, M A; Taylor, J C
Experimental steroid-induced osteonecrosis in adult rabbits with hypersensitivity vasculitis.
Osteonecrosis (ON) was experimentally induced in rabbits by employing a combined protocol of hypersensitivity vasculitis and administration of high-dose corticosteroids. Thirty-five adult rabbits were used: five were injected twice with horse serum (Group A), five were injected three times with methylprednisolone acetate (Group B), 20 were treated with a combination of horse serum and methylprednisolone acetate (Group C), and five were used as a control (Group D). Both femurs of each rabbit were obtained one to five weeks after the final treatment and were histologically examined. There was no evidence of ON in Groups A, B, and D, whereas vasculitis was prominent in the femurs of Group A rabbits. In Group C, 14 of 20 specimens (70%) showed histologic evidence of ON in the femoral metaphysis: seven showed marrow necrosis and seven marrow and trabecular necrosis. Intramedullary hemorrhage was detected in eight animals. All specimens that showed ON or marrow necrosis revealed arteriopathy (i.e., severe damage to the vascular wall structure of arterioles). These findings were similar to those observed in early ON of clinical materials. The authors conclude that arteriopathy plays an important role in the pathogenesis of ON. PMID:1555358
Matsui, M; Saito, S; Ohzono, K; Sugano, N; Saito, M; Takaoka, K; Ono, K
T cell receptor (TCR) V gene usage in patients with systemic necrotizing vasculitis.
Wegener's granulomatosis (WG) and polyarteritis nodosa (PAN) are systemic necrotizing vasculitides of unknown etiology. These disorders run a fatal course if untreated. T lymphocytes are implicated in the pathogenesis of WG, since they have been found to infiltrate affected organs, and sIL-2R correlates with disease activity. To elucidate further the role of T cells in necrotizing vasculitis, we have used a panel of 12 TCR V-specific MoAbs to investigate the number of cells expressing certain V alpha and V beta gene segments in the CD4+ and CD8+ subsets of altogether 11 patients with WG or PAN. In the group of patients, we found abnormal expansions of T cells using particular TCR V alpha or V beta gene products. These T cell expansions were more numerous, of a dramatically higher magnitude, and frequently more often found in the CD4 subset, compared with T cell expansions identified in healthy individuals. In long-term studies of the T cell expansions for up to 18 months, a heterogeneous pattern was revealed, with no obvious correlation to clinical features such as disease activity or treatment. Studies of TCR V gene usage in this group of patients may help in understanding the pathogenesis of necrotizing vasculitis, and in the identification of unknown antigens, and may open the possibility to a highly selective immunotherapy by targeting disease-mediating T cells. PMID:7648706
Giscombe, R; Grunewald, J; Nityanand, S; Lefvert, A K
Wet Shape Memory Alloy Actuators for Active Vasculated Robotic Flesh Stephen A. Mascaro and H is presented where Shape Memory Alloy (SMA) wires are embedded within artificial "blood vessels." Fluid flowing Memory Alloys, which are thin enough to fit inside of an artificial blood vessel while generating large
Mascaro, Stephen A.
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... do not heal Muscles and joints: Joint pain Leg pain Muscle contractions Wasting away of muscles Brain and nervous system: Pain, numbness , tingling in an arm, leg, or other body area Weakness of an arm, ...
Simultaneous Automated Screening and Confirmatory Testing for Vasculitis-Specific ANCA
Anti-neutrophil cytoplasmic antibodies (ANCA) are the serological hallmark of small vessel vasculitis, so called ANCA-associated vasculitis. The international consensus requires testing by indirect immunofluorescence (IIF) on human ethanol-fixed neutrophils (ethN) as screening followed by confirmation with enzyme-linked immunosorbent assays (ELISAs). This study evaluates the combination of cell- and microbead-based digital IIF analysis of ANCA in one reaction environment by the novel multiplexing CytoBead technology for simultaneous screening and confirmatory ANCA testing. Sera of 592 individuals including 118 patients with ANCA-associated vasculitis, 133 with rheumatoid arthritis, 49 with infectious diseases, 77 with inflammatory bowel syndrome, 20 with autoimmune liver diseases, 70 with primary sclerosing cholangitis and 125 blood donors were tested for cytoplasmic ANCA (C-ANCA) and perinuclear ANCA (P-ANCA) by classical IIF and ANCA to proteinase 3 (PR3) and myeloperoxidase (MPO) by ELISA. These findings were compared to respective ANCA results determined by automated multiplex CytoBead technology using ethN and antigen-coated microbeads for microbead immunoassays. There was a good agreement for PR3- and MPO-ANCA and a very good one for P-ANCA and C-ANCA by classical and multiplex analysis (Cohen's kappa [?]?=?0.775, 0.720, 0.876, 0.820, respectively). The differences between classical testing and CytoBead analysis were not significant for PR3-ANCA, P-ANCA, and C-ANCA (p<0.05, respectively). The prevalence of confirmed positive ANCA findings by classical testing (IIF and ELISA) compared with multiplex CytoBead analysis (IIF and microbead immunoassay positive) resulted in a very good agreement (??=?0.831) with no significant difference of both methods (p?=?0.735). Automated endpoint-ANCA titer detection in one dilution demonstrated a very good agreement with classical analysis requiring dilution of samples (??=?0.985). Multiplexing by CytoBead technology can be employed for simultaneous screening and quantitative confirmation of ANCA. This novel technique provides fast and cost-effective ANCA analysis by automated digital IIF for the first time. PMID:25225805
Sowa, Mandy; Grossmann, Kai; Knutter, Ilka; Hiemann, Rico; Rober, Nadja; Anderer, Ursula; Csernok, Elena; Bogdanos, Dimitrios P.; Borghi, Maria Orietta; Meroni, Pier Luigi; Schierack, Peter; Reinhold, Dirk; Conrad, Karsten; Roggenbuck, Dirk
Necrotizing vasculitis in a case of disseminated neonatal herpes simplex infection.
A term newborn suffered disseminated herpes simplex virus (HSV) type II infection five days after cesarean section delivery for fetal distress. The mother had no history or evidence of herpetic lesions; the father had a history of genital herpetic lesions. The infant's terminal course was dominated by disseminated intravascular coagulation (DIC) with hepatic and renal failure. Microscopic examination revealed a necrotizing vasculitis of small and medium-sized lung and peripancreatic arteries. Nuclear inclusions characteristic of HSV were found in these arteries, as well as in the adrenal parenchyma, spleen, and lymph node; electron microscopy confirmed replication of virus within the arterial endothelial cells. The mechanism of arterial damage in severe herpetic infection contrasts with the immune-complex mechanism postulated for other viral vasculitides. Direct, virally induced arterial damage resulting in exposure of collagen may set the stage of DiC, a commonly fatal complication of this disease. PMID:6895828
Phinney, P R; Fligiel, S; Bryson, Y J; Porter, D D
Localized chronic fibrosing vasculitis in a tattoo: a unique adverse tattoo reaction.
Decorative tattoos are associated with a variety of adverse cutaneous reactions. We describe a unique fibrosing vasculitic reaction to red tattoo ink. The histopathology was similar to that in localized chronic fibrosing vasculitis (LCFV), but sharply limited to sites of red tattoo ink injection and associated with florid verrucoid epidermal hyperplasia. LCFV has been described in a broad variety of slowly progressive disorders with a firm consistency such as erythema elevatum diutinum, plasma cell granuloma, granuloma faciale, and IgG4-associated sclerosing diseases. It has been hypothesized that LCFV is the result of maladaptive immune reaction with failure to clear the causative antigen. To the best of our knowledge, this is the first case of LCFV associated with tattoo. We speculate on the implications our case holds for the pathogenesis of LCFV. PMID:24736671
Deeken, Audrey; Jefferson, Julie; Hawkinson, Dana; Fraga, Garth R
Retinal vasculitis in two pediatric patients with systemic lupus erythematosus: a case report
We report two pediatric female patients with systemic lupus erythematosus (SLE) who presented with decreased vision. Both patients were found to have retinal vasculitis and occlusive disease. The first patient also presented with vitreous hemorrhage and later non-arteritic ischemic optic neuropathy. She was treated with panretinal photocoagulation and steroid therapy and later in her disease course was treated with rituximab and cyclophosphamide. Her vision remained decreased. The second patient was treated with rituximab and monthly cyclophosphamide infusions early in her disease course, and her vision improved dramatically. The difference in the presentations and outcomes of these two pediatric patients with SLE highlights the spectrum of severity of SLE retinopathy. We suggest that early recognition of disease and early intervention with B-cell depletion therapy in addition to a traditional cytotoxic agent should be considered in pediatric patients with SLE and occlusive retinopathy. PMID:23734963
Magnetic resonance imaging of skeletal muscle involvement in limb restricted vasculitis
Objective: To describe the MRI findings in the legs of three patients with limb restricted vasculitides (two PAN, one isolated vasculitis of the skeletal muscle) with histologically established muscle involvement. Methods: MRI was carried out on calf muscles and T2 weighted images, unenhanced T1 weighted images, and STIR sequences were obtained. Results: Muscle damage resulted in oedema-like changes on MRI characterised by hyperintense signals in T2 weighted and slow tau inversion recovery (STIR) sequences and normal unenhanced T1 weighted sequences of one or several leg muscles. Conclusions: MRI should be considered a useful complementary examination that might facilitate the recognition of limb restricted vasculitides, and possibly indicate the site for muscle biopsy. It could also be useful in monitoring the course of the disease. Future studies should also evaluate MRI for systemic PAN or other systemic vasculitides with muscle involvement. PMID:12429545
Gallien, S; Mahr, A; Rety, F; Kambouchner, M; Lhote, F; Cohen, P; Guillevin, L
The clinical spectrum and outcome of necrotizing vasculitis were evaluated in a retrospective study in order to assess: (1) the clinical expression and evolution of the disease; (2) the response to several therapeutic approaches based on major events (organ involvement causing disability or death); (3) the immunogenetic background of patients. Sixty-six Greek patients fulfilling the ACR criteria for the diagnosis of vasculitis entered the study. Thirty-seven were diagnosed with Wegener's granulomatosis (WG), 22 with polyarteritis nodosa (PAN) and seven with Churg-Strauss syndrome (CSS). The demographic characteristics of patients with WG and PAN were similar. Cutaneous manifestations, gastrointestinal and peripheral nervous system involvement occurred more often in patients with PAN, whereas pulmonary and upper respiratory tract involvement, renal, ear abnormalities and fever were more frequent in patients with WG. Muscle weakness and asthma were found exclusively in patients with PAN and CSS, respectively, while the presence of classic-antineutrophil cytoplasmic antibodies (c-ANCA) characterized WG patients. Hepatitis B surface antigen (HBsAg) was found in 22% of PAN patients. No significant differences were detected when comparing the PAN and WG groups with respect to the first major event (log-rank P = 0.50) with and without potential confounders (age, gender, therapy or c-ANCA). For WG patients, a statistically significant difference was found on different routes of administration of cyclophosphamide (oral vs pulse) (P = 0.006). Regarding the HLA antigens, an increased frequency of DR1 (26.9% vs 10.3%, P = 0.057) in WG and the absence of DR3 in patients with PAN and CSS were noted. It appears that although the immunogenetic background and the clinical expression of the diseases differ, the response to treatment as well as the evolution and the survival rate of these patients are similar in the two groups. PMID:9374922
Boki, K A; Dafni, U; Karpouzas, G A; Papasteriades, C; Drosos, A A; Moutsopoulos, H M
Coagulation and Fibrinolysis Index Profile in Patients with ANCA-Associated Vasculitis
Background Previous studies observed the high prevalence of venous thromboembolism in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The current study analyzed the coagulation and fibrinolysis index profile in AAV patients. Methods The current study recruited 321 AAV patients in active stage and 78 AAV patients in quiescent stage. Coagulation and fibrinolysis index profiles in these AAV patients were analysed, and their associations with various clinical and pathological parameters were further investigated. Results The circulating levels of D-dimer, fibrin degradation products and platelet count were significantly higher in AAV patients in active stage compared with those in remission [0.8 (0.4, 1.5) mg/L vs. 0.28 (0.2, 0.55) mg/L, P<0.05; 5.6 (5.0, 10.0) mg/L vs. 1.9 (1.2, 2.8) mg/L, P<0.05; 269�127�109/L vs. 227�80�109/L, P<0.05, respectively]. Among the 321 AAV patients in active stage, compared with patients with normal levels of D-dimer, patients with elevated D-dimer levels had significantly higher levels of initial serum creatinine, erythrocyte sedimentation rate, C reactive protein and the Birmingham Vasculitis Activity Scores (P?=?0.014, P<0.001, P<0.001, P?=?0.002, respectively). Moreover, correlation analysis showed that the levels of D-dimer correlated with erythrocyte sedimentation rate and C reactive protein levels (r?=?0.384, P<0.001; r?=?0.380, P<0.001, respectively). Conclusion Patients with active AAV are in hypercoagulable states, and circulating levels of D-dimer are associated with disease activity of AAV. PMID:24842719
Ma, Tian-Tian; Huang, Yi-Min; Wang, Chen; Zhao, Ming-Hui; Chen, Min
Flushing and Urticarial Syndromes Presenting as Anaphylaxis
\\u000a Flushing, urticaria, and angioedema are clinical findings that are commonly associated with anaphylaxis. Flushing can be quite\\u000a dramatic but is less common in anaphylaxis than are urticaria and angioedema, symptoms that are commonly mentioned together\\u000a as a single symptom,�urticaria\\/angioedema.� Differentiation of �dry flushing,� due to circulating agents acting directly on\\u000a smooth muscle, from �wet flushing,� due to neurogenic triggers from
Joseph H. Butterfield
PR3 ANCA is a classic marker of granulomatosis with polyangiitis (GPA). There have been several recent reports of increased prevalence of PR3ANCA in ulcerative colitis (UC) patients, the clinical implication of which is not well defined. We are reporting a case of 27-year-old Caucasian male with 14-year history of UC presenting with unilateral proptosis, conjunctival congestion, and chemosis who developed acute hemiparesis within three days of hospital admission, followed by rapid neurological deterioration correlating with brain imaging findings. Serologically he had atypical PANCA with high PR3 antibody titer with a negative infectious workup. His cerebral angiogram was normal but the brain biopsy showed necrotizing vasculitis. He was diagnosed with PR3 ANCA mediated cerebral and orbital vasculitis associated with UC. Treatment was initiated with high dose steroids, plasmapheresis, and cyclophosphamide. He improved significantly with residual left hemiparesis. PMID:25097791
Uchiyama, Robert C.
In systemic vasculitis, interactions be- tween antineutrophil cytoplasm autoanti- bodies (ANCAs) and neutrophils initiate endothelial and vascular injury. ANCAs directed against either myeloperoxidase (MPO) or proteinase 3 (PR3) can activate cytokine-primed neutrophils by binding cell surface-expressed MPO or PR3, with the concurrent engagement of Fcg recep- tors (FcgR). Because roles for phospho- lipase D (PLD) and phosphatidylinositol 3 kinase (PI3K)
Anne Ben-Smith; Stephen K. Dove; Ashley Martin; Michael J. O. Wakelam; Caroline O. S. Savage
Introduction��Primary central nervous system (CNS) vasculitis of childhood is a rare disorder. The most common signs and symptoms are acute\\u000a severe headache and focal neurologic deficit. It should be suspected in children who have an acquired neurologic deficit that\\u000a remains unexplained after an initial basic evaluation. Diagnosis usually depends on brain magnetic resonance imaging and conventional\\u000a angiography of cerebral vasculature.
Eren Kale �ekinmez; Nurcan Cengiz; ?lknur Erol; Osman K?z?lk?l?�; Yasemin Uslu
Objective. Rituximab, a chimeric anti-CD20 monoclonal antibody, has been shown to be quite effective in the treatment of immune disorders resulting from autoantibodies. We prospectively studied the long-term effects of rituximab in 10 patients with anti-neutrophil cytoplasmic antibody (ANCA)-positive vasculitis refractory to conventional therapy (n^3) or in second or subsequent relapse (n^7). Methods. The median age of patients was 53
R. Stasi; E. Stipa; G. Del Poeta; S. Amadori; A. C. Newland; D. Provan
Pulmonary involvement in ANCA-associated vasculitis from the view of the pulmonologist.
Microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) are conditions classified under the general heading of antinuclear cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). Lung lesion is a very common and important clinical feature in AAV. In MPA, diffuse alveolar hemorrhage and pulmonary fibrosis (PF) are the most frequent manifestations. High-resolution computed tomography (HRCT) chest findings associated with MPA in PF patients demonstrate a high frequency of usual interstitial pneumonia (UIP), fibrotic-nonspecific interstitial pneumonia (F-NSIP), and combined PF and emphysema (CPFE) pattern with honeycombing, traction bronchiectasis, ground-glass opacity, and emphysema. In most of these cases, the histologic pattern of PF has been classified as UIP and/or fibrotic NSIP. In addition, a high incidence of histological findings, such as extensive interstitial fibrosis, lymphoid hyperplasia, and bronchiolitis, are characteristics observed in PF associated with collagen vascular diseases and which are not observed in idiopathic PF (IPF). In some cases, PF precedes the development of MPA. Indeed, there are some cases of pulmonary-limited MPA in this group. Therefore, clinicians should be aware of MPA as an underlying feature of PF in order to avoid overlooking and misdiagnosing this condition as IPF. The median survival time (MST) in UIP pattern/MPA is comparable with that of IPF. In GPA, almost all patients have either upper airway or lower respiratory tract lesions. Solitary or multiple nodules (frequently cavitated) and masses are the most common findings on chest images. Asthma is a cardinal symptom of Churg-Straus syndrome, often preceded by allergic rhinitis. To induce remission, a severity-based regimen was given to patients according to the appropriate protocol of the Japanese patients with myeloperoxidase (MPO)-ANCA-associated vasculitis (JMAAV) study group: low-dose corticosteroid and, if necessary, cyclophosphamide or azathioprine in patients with mild form; high-dose corticosteroid and cyclophosphamide in those with severe form; severe-form regimen plus plasmapheresis in those with the most severe form. PMID:23188194
Homma, Sakae; Suzuki, Aika; Sato, Keita
Background The purpose of the study is to describe the clinical course and treatment of idiopathic retinitis, vasculitis, aneurysms and neuroretinitis. The study utilized non-randomized, retrospective and interventional case series. The eight eyes of six patients were analysed. Testing included wide fluorescein angiography, indocyanine green angiography and systemic evaluation. Treatment involved observation, panretinal laser photocoagulation (PRP) for peripheral retinal ischemia, grid laser for macular oedema and focal laser on the macroaneurysms. The main outcome measures were initial visual acuity (VA), initial stage at diagnosis, clinical course, surgical intervention, final VA, final stage and complications of disease. Results Five out of eight eyes with retinal ischemia in more than two quadrants that were treated with PRP and grid laser for macular oedema maintained excellent VA and demonstrated no progression of retinal ischemia during follow-up. The two eyes which exhibited retinal ischemia in less than two quadrants and macular oedema were treated with grid laser and focal laser on the macroaneurysms, but did not undergo PRP. VA improved by two lines of the Snellen chart, and there was no progression of retinal ischemia during the 3 and 4 years of follow-up. One eye with neither retinal ischemia nor macular oedema was not treated, and the clinical picture remained stable during the follow-up. Conclusion Early PRP may be considered in the presence of angiographic evidence of peripheral retinal non-perfusion. However, treatment could be withheld until the patient develops retinal ischemia in more than two quadrants. PMID:23514018
Prior to the 1970s, severe cases of antineutrophil cytoplasmic antibody associated vasculitis (AAV) were thought to be invariably fatal. However, the use of cyclophosphamide-based treatment regimens fundamentally altered disease outcomes, transforming AAV into a manageable, chronic illness. Despite the tremendous success of cyclophosphamide in the treatment of AAV, there remained a need for alternative therapies, due to high rates of treatment failures and significant toxicities. In recent years, with the introduction of targeted biologic response modifiers into clinical practice, many have hoped that the treatment options for AAV could be expanded. Rituximab, a chimeric monoclonal antibody directed against the B-lymphocyte protein CD20, has been the most successful biologic response modifier to be used in AAV. Following the first report of its use in AAV in 2001, experience with rituximab for treatment of AAV has rapidly expanded. Rituximab, in combination with glucocorticosteroids, is now well established as a safe and effective alternative to cyclophosphamide for remission induction for severe manifestations of granulomatosis with polyangiitis and microscopic polyangiitis. In addition, initial experiences with rituximab for remission maintenance in these diseases have been favorable, as have experiences for remission induction in eosinophilic granulomatosis with polyangiitis. PMID:24688606
Clain, Jeremy M.; Cartin-Ceba, Rodrigo; Fervenza, Fernando C.
Genetic Susceptibility to ANCA-Associated Vasculitis: State of the Art
ANCA-associated vasculitis (AAV) is a group of disorders that is caused by inflammation affecting small blood vessels. Both arteries and veins are affected. AAV includes microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) renamed from Wegener�s granulomatosis, and eosinophilic granulomatosis with polyangiitis (EGPA), renamed from Churg�Strauss syndrome. AAV is primarily due to leukocyte migration and resultant damage. Despite decades of research, the mechanisms behind AAV disease etiology are still not fully understood, although it is clear that genetic and environmental factors are involved. To improve the understanding of the disease, the genetic component has been extensively studied by candidate association studies and two genome-wide association studies. The majority of the identified genetic AAV risk factors are common variants. These have uncovered information that still needs further investigation to clarify its importance. In this review, we summarize and discuss the results of the genetic studies in AAV. We also present the novel approaches to identifying the causal variants in complex susceptibility loci and disease mechanisms. Finally, we discuss the limitations of current methods and the challenges that we still have to face in order to incorporate genomic and epigenomic data into clinical practice.
Bonatti, Francesco; Reina, Michele; Neri, Tauro Maria; Martorana, Davide
A 1.5-year-old female Mississippi sandhill crane (Grus canadensis pulla) was presented and managed for a polyarthritis of the intertarsal and tarsophalangeal articulations. Results of aerobic bacterial cultures, Mycoplasma species culture, and polymerase chain reaction testing of articular fluid did not identify any causative organisms. Results of radiographs and cytologic examination of articular fluid were consistent with an inflammatory, nonerosive polyarthritis. The arthritis did not improve with systemic anti-inflammatory and antibiotic treatment and with joint lavage. A large necrotic granulomatous mass was detected on the right shoulder area from which Staphylococcus aureus and Enterococcus species were isolated as opportunistic pathogens. Two days after surgical resection of the mass, the distal polyarthritis resolved. Histopathologic examination of the mass was consistent with granulomatous vasculitis with abscess formation of unknown origin. In this crane, the unresponsiveness to standard therapy, the presence of an infected and inflammatory mass, and the resolution of the polyarthritis after the resection of the mass strongly supported a diagnosis of reactive immune-mediated nonerosive polyarthritis. Analysis of this case suggests that immune-mediated idiopathic arthritis should be a differential diagnosis of distal polyarthritis in cranes and that an inciting source remote from the joints should be investigated in case of lack of response to standard therapy. PMID:24640933
MacLean, Robert; Beaufr�re, Hugues; Heggem-Perry, Brittany; Field, Cara; Garner, Michael
Names influence how something is perceived. Diagnostic terms (diagnoses) are the names of diseases that are usually derived either from some distinctive characteristic of the disease or include an eponym recognizing someone who elucidated the disease. No matter how logical and appropriate a name may be, if it is not usable and used it is of no lasting value. This brief commentary focuses on the nomenclature of systemic vasculitides, and uses as a prime example Wegener's granulomatosis, which has been renamed recently �granulomatosis with polyangiitis�, in part because of concerns about the suitability of Friedrich Wegener as the source of an eponym. The most distinctive pathological feature of Wegener's granulomatosis is multi-focal necrotizing inflammation that has long been called granulomatosis. The systemic variant of Wegener's granulomatosis also is characterized by inflammation in many different vessels or different types, i.e. polyangiitis. Thus, granulomatosis with polyangiitis is a very appropriate alternative term for Wegener's granulomatosis. This term also is in accord with the name for a closely related vasculitis, i.e. microscopic polyangiitis. Terms that indicate aetiology and pathogenesis, when known, are useful to include in names for diseases (diagnoses). Anti-neutrophil cytoplasmic autoantibodies specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA) are implicated in the cause of granulomatosis with polyangiitis and thus also should be specified in the diagnosis (e.g. PR3-ANCA-positive granulomatosis with polyangiitis or MPO-ANCA-positive microscopic polyangiitis). As our understanding of the clinical manifestations, pathogenesis and aetiology of vasculitides change over time, the names and approaches for diagnosing these diseases will change accordingly. PMID:21447122
Jennette, J C
Objective Immunosuppression is cornerstone treatment of antineutrophil cytoplasmic antibody associated vasculitis (AAV) but is later complicated by infection, cancer, cardiovascular and chronic kidney disease. Caveolin-1 is an essential structural protein for small cell membrane invaginations known as caveolae. Its functional role has been associated with these complications. For the first time, caveolin-1 (CAV1) gene variation is studied in AAV. Methods CAV1 single nucleotide polymorphism rs4730751 was analysed in genomic DNA from 187 white patients with AAV from Birmingham, United Kingdom. The primary outcome measure was the composite endpoint of time to all-cause mortality or renal replacement therapy. Secondary endpoints included time to all-cause mortality, death from sepsis or vascular disease, cancer and renal replacement therapy. Validation of results was sought from 589 white AAV patients, from two European cohorts. Results The primary outcome occurred in 41.7% of Birmingham patients. In a multivariate model, non-CC genotype variation at the studied single nucleotide polymorphism was associated with increased risk from: the primary outcome measure [HR 1.86; 95% CI: 1.14-3.04; p=0.013], all-cause mortality [HR:1.83; 95% CI: 1.02-3.27; p=0.042], death from infection [HR:3.71; 95% CI: 1.28-10.77; p=0.016], death from vascular disease [HR:3.13; 95% CI: 1.07-9.10; p=0.037], and cancer [HR:5.55; 95% CI: 1.59-19.31; p=0.007]. In the validation cohort, the primary outcome rate was far lower (10.4%); no association between genotype and the studied endpoints was evident. Conclusions The presence of a CC genotype in Birmingham is associated with protection from adverse outcomes of immunosuppression treated AAV. Lack of replication in the European cohort may have resulted from low clinical event rates. These findings are worthy of further study in larger cohorts. PMID:23894397
Chand, Sourabh; Holle, Julia U.; Hilhorst, Marc; Simmonds, Matthew J.; Smith, Stuart; Kamesh, Lavanya; Hewins, Peter; McKnight, Amy Jayne; Maxwell, Alexander P.; Cohen Tervaert, Jan Willem; Wieczorek, Stefan; Harper, Lorraine; Borrows, Richard
We present the case of an 11-year-old boy presenting with haemoptysis, dyspnoea and weight loss as a manifestation of isolated pulmonary vasculitis, leading to pulmonary hypertension. He also appeared to have a longstanding dural venous sinus thrombosis. This rare presentation, especially in childhood, might represent a case of the seldomly reported Hughes-Stovin syndrome. The patient achieved remission after therapy with cyclophosphamide pulses and high-dose steroids. Based on the presented case and review of the literature, we propose that this syndrome might be a variant of polyarteritis nodosa. This report highlights diagnostic issues and describes a successful treatment regimen. PMID:21816089
Patients with clinical suspicion of large-vessel vasculitis (LVV) may present with nonspecific signs and symptoms and increased inflammatory parameters and may remain without diagnosis after routine diagnostic procedures. Both the nonspecificity of the radiopharmaceutical 18F-FDG and the synergy of integrating functional and anatomical images with PET/CT offer substantial benefit in the diagnostic work-up of patients with clinical suspicion for LVV. A negative temporal artery biopsy, an ultrasonography without an arterial halo, or a MRI without aortic wall thickening or oedema do not exclude the presence of LVV and should therefore not exclude the use of 18F-FDG PET/CT when LVV is clinically suspected. This overview further discusses the notion that there is substantial underdiagnosis of LVV. Late diagnosis of LVV may lead to surgery or angioplasty in occlusive forms and is often accompanied by serious aortic complications and a fatal outcome. In contrast to the American College of Rheumatology 1990 criteria for vasculitis, based on late LVV effects like arterial stenosis and/or occlusion, 18F-FDG PET/CT sheds new light on the classification of giant cell arteritis (GCA) and Takayasu arteritis (TA). The combination of these observations makes the role of 18F-FDG PET/CT in the assessment of patients suspected for having LVV promising. PMID:25328890
Balink, H.; Bennink, R. J.; van Eck-Smit, B. L. F.; Verberne, H. J.
Background: This case report represents one of the estimated 17,000 aneurysms clipped annually in the United States, often with nickel-containing clips. The authors highlight the development of life-threatening allergic vasculitis in a 33-year-old woman after aneurysm clipping. Case Description: After suffering subarachnoid hemorrhage, the patient had coil embolization at another facility for rupture of a right internal carotid artery (ICA) aneurysm. An incidental finding, an unruptured left posterior communicating artery aneurysm unamenable to coiling, was then successfully clipped via a left pterional craniotomy. Arriving in our emergency department 11 days later, she progressively declined during the next weeks, facing deteriorating clinical status (i.e. seizures) and additional infarctions in the left frontal lobe, midline shift, and new infarctions in the bilateral frontal lobe, right sylvian, right insular regions, and posterior cerebral artery distribution. During decompressive surgery, biopsy findings raised the possibility of lymphocytic vasculitis; consultations with rheumatology, allergy, and immunology specialists identified that our patient had a nickel allergy. After reoperation to replace the nickel-containing clip with one of a titanium alloy, the patient had an uncomplicated postoperative course and was discharged 6 days later to a rehabilitation facility. Conclusions: Nickel-related allergies are more common than appreciated, affecting up to 10% of patients. Fortunately, severe reactions are rare; nevertheless, vascular neurosurgeons should be aware of this potential complication when using cobalt alloy aneurysms clips. The use of titanium alloy clips eliminates this risk. PMID:25071940
Grande, Andrew; Grewal, Sanjeet; Tackla, Ryan; Ringer, Andrew J.
Introduction We investigated the clinical and serological features of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in Japan using data from a nationwide, prospective, inception cohort study. Methods In total, 156 Japanese patients with newly diagnosed AAV were classified according to the European Medicines Agency (EMEA) algorithm with exploratory surrogate markers for AAV-related non-granulomatous pulmonary lesions, predefined as alveolar haemorrhage and interstitial lung disease (ILD), and their clinical and serological features were evaluated. Results Using the EMEA algorithm, we identified 14 patients (9.0%) with eosinophilic granulomatosis with polyangiitis (EGPA), 33 (21.2%) with granulomatosis with polyangiitis (GPA), 78 (50.0%) with microscopic polyangiitis and renal-limited vasculitis (MPA/RLV), and 31 (19.9%) with unclassifiable vasculitis. The average ages of patients with EGPA (male/female, 5/9), GPA (12/21), and MPA/RLV (35/43) and unclassifiable (9/22) were 58.0, 63.6, 71.1, and 70.6�years, respectively. Myeloperoxidase (MPO)-ANCA and proteinase-3 ANCA positivity was 50.0% and 0% for EGPA, 54.6% and 45.5% for GPA, 97.4% and 2.6% for MPA/RLV, and 93.5% and 3.2% for unclassifiable, respectively. According to the Birmingham Vasculitis Activity Score (BVAS), cutaneous (71.4%) and nervous system (92.9%) manifestations were prominent in EGPA and ear, nose, and throat manifestations (84.9%) and chest manifestations (66.7%) in GPA. Renal manifestations developed frequently in MPA/RLV (91.0%) and GPA (63.6%). The average serum creatinine levels were 0.71�mg/dL for EGPA, 1.51�mg/dL for GPA, 2.46�mg/dL for MPA/RLV, and 0.69�mg/dL for unclassifiable. The percentages of patients with ILD were 14.3% for EGPA, 9.0% for GPA, 47.4% for MPA/RLV, and 61.3% for unclassifiable. Patients with ILD (n?=?61) had significantly lower BVAS (P?=?0.019) with fewer ear, nose, and throat and cardiovascular manifestations than patients without ILD (n?=?95). Conclusions MPO-ANCA-positive MPA/RLV is the most common form of AAV in Japanese patients, and one-half of patients with GPA were positive for MPO-ANCA. ILD is an important clinical manifestation in Japanese patients with AAV. Unclassifiable vasculitis with MPO-ANCA positivity and ILD may represent a novel variant of MPA. Trial Registration The University Hospital Medical Information Network Clinical Trials Registry: UMIN000001648. Registered 28 February 2009. PMID:24758294
Clinical outcomes in ANCA-associated vasculitis (AAV) and lupus nephritis have improved greatly with treatment regimens containing high-dose glucocorticoids and cyclophosphamide. However, with the use of these medications come significant adverse events, most notably infections, cytopenias, malignancies, and reproductive abnormalities. Multiple recent randomized controlled trials in AAV and lupus nephritis have compared cyclophosphamide-based regimens with agents such as rituximab, mycophenolate mofetil, and azathioprine, with the hope of providing better clinical outcomes with improved safety profiles. Although some of these newer regimens are now considered first-line treatments of these diseases, their adverse event profiles have been disappointingly similar to those of cyclophosphamide-based protocols. Physicians and patients should consider the adverse event profiles generated by these trials in the context of their extensive use in other patient populations, as well as available measures to prevent such events, when choosing the ideal regimen for an individual patient. PMID:24832093
Hogan, Jonathan; Avasare, Rupali; Radhakrishnan, Jai
Autologous stem cell transplantation (SCT) is increasingly used to treat autoimmune diseases (AD), in particular systemic sclerosis (SSc). Secondary autoimmune diseases are a known complication after autologous stem cell transplantations for any cause. A 43-year-old man had received an autologous stem cell transplantation for an aggressive diffuse cutaneous SSc. After mobilisation with cyclophosphamide and Granulocyte-Colony-Stimulating Factor stem cells were CD34-selected. The patient received a conditioning regimen with cyclophosphamide and Antithymocyte globulin. He had an excellent response with the modified Rodnan Skin Score decreasing from 34 to 3. One year and 4 months after SCT mild erythrocyturia without acanthocytes and proteinuria were seen for the first time on routine urinalysis. During the following year erythrocyturia increased to 131 erythrocytes /?l and protein excretion to 628 mg/g creatinine. At that time, acanthocytes of 25% finally could be detected. Due to the clearly nephritic constellation in urinalysis a renal biopsy was performed, which revealed mild global and focal-segmental sclerosing and focal-segmental proliferative glomerulonephritis without any signs of a IgA-nephropathy. The result was compatible with a renal manifestation of a small-vessel vasculitis. During the following laboratory workup ANCA of a perinuclear pattern with specificity for myeloperoxidase in high titers could be detected. Therefore the diagnosis of a p-ANCA-positive glomerulonephritis was established. As treatment, the patient received Rituximab, which turned out to be effective. We provide the first report of a patient who developed a p-ANCA-associated vasculitis after autologous stem cell transplantation for an autoimmune disease, namely systemic sclerosis. PMID:25005330
Schmalzing, Marc; Amann, Kerstin; Tony, Hans-Peter
Extraintestinal features may be observed in patients with ulcerative colitis (UC). We describe a 69-year-old woman who was initially diagnosed as having polymyalgia rheumatica (PMR). Prednisone was progressively tapered to complete discontinuation a year and a half after PMR diagnosis. However, at that time, she started to complain of asthenia, abdominal cramping and pain on the left side, weight loss and bloody diarrhoea. A colonoscopy confirmed a diagnosis of left-sided UC. She experienced several flares of the disease that required admission and treatment with high-dose corticosteroids and azathioprine. Colectomy was performed as the disease became refractory to these therapies. Four months after surgery, when the patient was not receiving any corticosteroid therapy, she started to feel dull and achy pain in the thighs along with claudication of the lower limbs. An 18F-fluorodeoxyglucosepositron emission tomography with CT (FDG PET/CT) disclosed an inflammatory process with mild-moderate diffuse increased metabolism in the thoracic aorta and markedly increased FDG uptake in the in the femoral and posterior tibial arteries on both sides. Treatment with the anti-TNF-alpha monoclonal antibody-adalimumab (40 mg every 2 weeks subcutaneously) along with prednisone (initial dose 15 mg/day) yielded rapid improvement of symptoms. Also, a new FDG PET/CT performed 4 months later disclosed marked decrease of FDG uptake in the involved arteries.This report emphasises the importance of suspecting the presence of large- and medium-vessel vasculitis in a patient with UC presenting with musculoskeletal features. It also highlights the beneficial effect of TNF-antagonists in vasculitis associated to UC. PMID:22640654
Bejerano, Carmen; Blanco, Ricardo; Gonz�lez-Vela, Carmen; P�rez-Mart�n, In�s; Martinez-Rodriguez, Isabel; Jimenez-Bonilla, Julio; Gonz�lez-Gay, Miguel A
In renal biopsy specimens from 15 patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated renal vasculitis, the infiltrating intraglomerular and interstitial leukocytes were localized and the adhesion molecules intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) and the cytokine interleukin-1? (IL-1?) were studied by an immunohistochemical method. Intraglomerular leukocytes were mainly macrophages (13.46 � 9.29 cells\\/glomerular cross-section) and, to a
Maria Pia Rastaldi; Franco Ferrario; Stefania Tunesi; Lin Yang; Giuseppe D'Amico
Classically presenting with multiple or single peripheral cytopenias of variable severity, the myelodysplastic syndromes may occasionally present with bizarre manifestations that confuse the clinical picture and result in significant delays in making the correct diagnosis. We describe the case of an elderly male patient whose presentation with prolonged unexplained fever coupled with cutaneous, pulmonary and other systemic features of inflammation was finally diagnosed as having a primary myelodysplastic syndrome with associated vasculitis after a delay of 4 years. PMID:22031795
Hassan, Imad Salah; Dar, Javeed
Circulating antibodies to myeloperoxidase (MPO) are associated primarily with pauci-immune glomerulonephritis and systemic vasculitis. Anti-MPO antibodies belong to a group of autoantibodies, anti-neutrophil cytoplasmic antibodies, that may play a pathogenic role in vasculitis. We have generated a human monoclonal anti-MPO antibody (E3-MPO) using peripheral blood lymphocytes from a patient with microscopic polyarteritis. Variable region gene analysis of E3-MPO showed that the VH region had 90% homology with the germ line gene VH4-21. E3-MPO was also shown to carry the 9G4 idiotope, which so far has been associated only with human antibodies that utilize the VH4-21 gene. The 9G4 idiotope was also expressed on anti-MPO antibodies in sera from the donor patient and from 4/7 additional patients with active, untreated vasculitis. The nucleotide sequences of both the variable heavy and light chains of E3-MPO showed evidence of an antigen-driven response. PMID:8698399
Longhurst, C; Ehrenstein, M R; Leaker, B; Stevenson, F K; Spellerberg, M; Chapman, C; Latchmen, D; Isenberg, D A; Cambridge, G
Levamisole-induced vasculitis is a relatively new entity in people who use cocaine. We describe a 44-year-old woman with a history of cocaine use who presented with a complaint of a painful rash of 2-3 month�s duration on her extremities, cheeks, nose, and earlobes. She had not experienced fever, weight loss, alopecia, dry eyes, oral ulcers, photosensitivity, or arthralgia. Examination revealed tender purpuric eruptions with central necrosis on her nose, cheeks, earlobes, and extremities. Laboratory investigations revealed neutropenia, an elevated erythrocyte sedimentation rate (ESR), presence of lupus anticoagulant, low complement component 3 (C3), and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). A urine toxicology screen was positive for cocaine, and gas chromatography�mass spectrometry was positive for levamisole. Skin biopsy showed leukocytoclastic vasculitis and small vessel thrombosis. Necrotic lesions of the nose led to its self-amputation. Large bullae on the lower extremities ruptured, leading to wound infection and extensive necrosis that required multiple surgical debridements. When necrosis progressed despite debridement, bilateral above-knee amputation of the legs was performed. Once new lesions stopped appearing, the patient was discharged home. Two months later, she had a recurrence related to cocaine use. To the best of our knowledge, this is only the second reported case of levamisole-induced vasculitis that required above-knee amputation. PMID:23186390
Levamisole-induced vasculitis is a relatively new entity in people who use cocaine. We describe a 44-year-old woman with a history of cocaine use who presented with a complaint of a painful rash of 2-3 month's duration on her extremities, cheeks, nose, and earlobes. She had not experienced fever, weight loss, alopecia, dry eyes, oral ulcers, photosensitivity, or arthralgia. Examination revealed tender purpuric eruptions with central necrosis on her nose, cheeks, earlobes, and extremities. Laboratory investigations revealed neutropenia, an elevated erythrocyte sedimentation rate (ESR), presence of lupus anticoagulant, low complement component 3 (C3), and presence of perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). A urine toxicology screen was positive for cocaine, and gas chromatography-mass spectrometry was positive for levamisole. Skin biopsy showed leukocytoclastic vasculitis and small vessel thrombosis. Necrotic lesions of the nose led to its self-amputation. Large bullae on the lower extremities ruptured, leading to wound infection and extensive necrosis that required multiple surgical debridements. When necrosis progressed despite debridement, bilateral above-knee amputation of the legs was performed. Once new lesions stopped appearing, the patient was discharged home. Two months later, she had a recurrence related to cocaine use. To the best of our knowledge, this is only the second reported case of levamisole-induced vasculitis that required above-knee amputation. PMID:23186390
Arora, Natasha Purai; Jain, Tania; Bhanot, Ravinder; Natesan, Suganthini Krishnan
We report a case of hydralazine-induced ANCA-associated glomerulonephritis with pulmonary hemorrhage. A 62-year-old Hispanic man with hypertension, who was being treated with hydralazine 100?mg three times a day for four and half years, presented to the hospital with severe anemia. He had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. CT scan of the chest revealed bilateral pulmonary ground-glass infiltrates. Transbronchial biopsy was consistent with pulmonary hemorrhage. Serologic tests showed high titer PR3 ANCA and, to a lesser extent, MPO ANCA. Kidney biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, without evidence of immune complex deposits. Hydralazine was discontinued and the patient was treated with corticosteroids and intravenous cyclophosphamide. At one-year follow-up, he had no symptoms and anemia had resolved. Kidney function improved dramatically. Serology showed undetectable PR3 ANCA and minimally elevated MPO ANCA. To our knowledge, hydralazine-associated PR3 ANCA has not been previously reported. The possibility of ANCA systemic vasculitis should be included in the differential diagnosis of any patient with hydralazine use and pulmonary renal syndrome. This is a potentially life threatening condition requiring prompt cessation of the drug and treatment with glucocorticoids and immunosuppression. PMID:25210633
Agarwal, Gaurav; Sultan, Ghayyath; Werner, Sherry L; Hura, Claudia
We report a case of hydralazine-induced ANCA-associated glomerulonephritis with pulmonary hemorrhage. A 62-year-old Hispanic man with hypertension, who was being treated with hydralazine 100?mg three times a day for four and half years, presented to the hospital with severe anemia. He had acute kidney injury and urinalysis showed proteinuria, dysmorphic RBCs, and rare RBC cast. CT scan of the chest revealed bilateral pulmonary ground-glass infiltrates. Transbronchial biopsy was consistent with pulmonary hemorrhage. Serologic tests showed high titer PR3 ANCA and, to a lesser extent, MPO ANCA. Kidney biopsy revealed focal segmental necrotizing glomerulonephritis with crescents, without evidence of immune complex deposits. Hydralazine was discontinued and the patient was treated with corticosteroids and intravenous cyclophosphamide. At one-year follow-up, he had no symptoms and anemia had resolved. Kidney function improved dramatically. Serology showed undetectable PR3 ANCA and minimally elevated MPO ANCA. To our knowledge, hydralazine-associated PR3 ANCA has not been previously reported. The possibility of ANCA systemic vasculitis should be included in the differential diagnosis of any patient with hydralazine use and pulmonary renal syndrome. This is a potentially life threatening condition requiring prompt cessation of the drug and treatment with glucocorticoids and immunosuppression. PMID:25210633
Sultan, Ghayyath; Werner, Sherry L.; Hura, Claudia
Palisaded neutrophilic granulomatous dermatitis (PNGD) is a rare dermatologic condition which shows various clinical and histopathological features. Although the PNGD lesions have been suggested to begin as leukocytoclastic vasculitis (LCV), there is still insufficient clinicopathological information in the reported cases of PNGD in acute stage with LCV. The relationship between PNGD and interstitial granumatous dermatis (IGD) also remains unclear. This report presents the case of a 60-year-old female patient with multiple erythematous nodules on the extremities. She had no underlying systemic disease detected to date, although transient, abnormal liver function tests were seen. The histopathological examination of an erythematous nodule revealed the features of PNGD in the acute stage. The patient presented the characteristic features of LCV including palisaded granulomatous pattern, and the interstitial granulomatous pattern was seen together, suggesting that PNGD with LCV can show an interstitial granulomatous pattern. The present case also suggested that PNGD in the acute stage with LCV tends to clinically manifest as erythematous nodules on the extremities and histopathologically shows a remarkable papillary edema and an extensive fibrin deposition in and around the vessel wall. PNGD may be associated with transient liver dysfunction. PMID:19903215
Misago, Noriyuki; Shinoda, Yohsuke; Tago, Masaki; Narisawa, Yutaka
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) treatment strategy is based on immunosuppressive agents. Little information is available concerning mycophenolic acid (MPA) and the area under the curve (AUC) in patients treated for AAV. We evaluated the variations in pharmacokinetics for MPA in patients with AAV and the relationship between MPA-AUC and markers of the disease. MPA blood concentrations were measured through the enzyme-multiplied immunotechnique (C(0), C(30), C(1), C(2), C(3), C(4), C(6) and C(9)) to determine the AUC. Eighteen patients were included in the study. The median (range) MPA AUC(0-12) was 50�55 (30�9-105�4) mg/h/l. The highest coefficient of determination between MPA AUC and single concentrations was observed with C(3) (P?0�0001) and C(2) (P?0�0001) and with C(4) (P?0�0005) or C(0) (P?0�001). Using linear regression, the best estimation of MPA AUC was provided by a model including C(30), C(2) and C(4): AUC?=?8�5?+?0�77 C(30) +?4�0 C(2) +?1�7 C(4) (P?0�0001). Moreover, there was a significant relationship between MPA AUC(0-12) and lymphocyte count (P?0�01), especially CD19 (P?0�005), CD8 (P?0�05) and CD56 (P?0�05). Our results confirm the interindividual variability of MPA AUC in patients treated with MMF in AAV and support a personalized therapy according to blood levels of MPA. PMID:24304103
Chaigne, B; Gatault, P; Darrouzain, F; Barbet, C; Degenne, D; Fran�ois, M; Szymanski, P; Rabot, N; Golea, G; Diot, E; Maillot, F; Lebranchu, Y; Nivet, H; Paintaud, G; Halimi, J-M; Guillevin, L; B�chler, M
Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases
Other Specified Inflammatory Disorders of Skin or Subcutaneous Tissue; Pyoderma Gangrenosum; Erosive Pustular Dermatosis of the Scalp; Sweet's Syndrome; Behcet's Disease; Bowel-associated Dermatosis-arthritis Syndrome; Pustular Psoriasis; Acute Generalized Exanthematous Pustulosis; Keratoderma Blenorrhagicum; Sneddon-Wilkinson Disease; IgA Pemphigus; Amicrobial Pustulosis of the Folds; Infantile Acropustulosis; Transient Neonatal Pustulosis; Neutrophilic Eccrine Hidradenitis; Rheumatoid Neutrophilic Dermatitis; Neutrophilic Urticaria; Still's Disease; Erythema Marginatum; Unclassified Periodic Fever Syndromes / Autoinflammatory Syndromes; Dermatitis Herpetiformis; Linear IgA Bullous Dermatosis; Bullous Systemic Lupus Erythematosus; Inflammatory Epidermolysis Bullosa Aquisita; Neutrophilic Dermatosis of the Dorsal Hands (Pustular Vasculitis); Small Vessel Vasculitis Including Urticarial Vasculitis; Erythema Elevatum Diutinum; Medium Vessel Vasculitis
Background Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) may present with pulmonary involvement ranging from mild to life-threatening disease such as diffuse alveolar hemorrhage. There is a paucity of information regarding morbidity outcomes for AAV subjects presenting with lung involvement. This study determines the relationship between disease activity and damage in these subjects using the Birmingham Vasculitis Activity Score v 3 (BVAS 3) and Vasculitis Damage Index (VDI) respectively. Results 151 patients with AAV were included with 59 presenting initially with pulmonary involvement. The initial BVAS scores recorded at time of diagnosis were positively correlated with the final VDI scores at 24�months (p?0.0001, rs?=?0.5871). No differences between BVAS and VDI scores were seen for both groups, however in the lung-involvement group only, BVAS scores were significantly higher at 6, 12 and 24�months whilst the VDI scores were significantly higher at 12 and 24�months. Subjects presenting with pulmonary involvement had an increased likelihood for cardiovascular (OR 1.31, 95% CI 0.89, 1.54; p?=?0.032) and renal (OR 1.32, 95% CI 1.22, 1.39; p?=?0.005) involvement. Subjects presenting with lung involvement with granulomatosis with polyangiitis and microscopic polyangiitis had 24-month VDI scores that were significantly higher (p?=?0.027, p?=?0.045), and more likely to develop pulmonary fibrosis (OR 1.79, 95% CI 1.48, 2.12; p?0.001). Conclusion AAV subjects with lung involvement at presentation had a higher disease activity and damage scores at 6, 12 and 24�months follow-up representing a considerable burden of disease despite improvement in overall survival due to the introduction of immunosuppressive therapy. PMID:24884372
American bison (Bison bison) are particularly susceptible to developing fatal sheep-associated malignant catarrhal fever (SA-MCF) caused by ovine herpesvirus-2 (OvHV-2), a ?-herpesvirus in the Macavirus genus. This generally fatal disease is characterized by lymphoproliferation, vasculitis, and mucosal ulceration in American bison, domestic cattle (Bos taurus), and other clinically susceptible species which are considered non-adapted, dead-end hosts. The pathogenesis and cellular tropism of OvHV-2 infection have not been fully defined. An earlier study detected OvHV-2 open reading frame 25 (ORF25) transcripts encoding the viral major capsid protein in tissues of bison with SA-MCF, and levels of viral transcript expression positively correlated with lesion severity. To further define the cellular tropism and replication of OvHV-2 infection in vascular lesions of bison, immunofluorescence studies were performed to identify cell type(s) expressing ORF25 protein within tissues. Cytoplasmic and not nuclear ORF25 protein was demonstrated in predominantly perivascular fibroblasts in six bison with experimentally-induced SA-MCF, and there was no evidence of immunoreactivity in vascular endothelium, smooth muscle, or infiltrating leukocytes. The cytoplasmic distribution of viral major capsid protein suggests that viral replication in perivascular fibroblasts may be abortive in this dead-end host. These findings provide a novel foundation for defining the pathogenesis of vasculitis in non-adapted hosts with SA-MCF. PMID:23953727
Nelson, Danielle D; Taus, Naomi S; Schneider, David A; Cunha, Cristina W; Davis, William C; Brown, Wendy C; Li, Hong; O'Toole, Donal; Oaks, J Lindsay
... as the aorta or the pulmonary arteries). Lung Function Tests Lung function tests measure how much air you can breathe ... your lungs deliver oxygen to your blood. Lung function tests can help your doctor find out whether ...
Central Nervous System Vasculitis
... headaches that last a long time strokes or transient ischemic attacks (�mini-strokes�) forgetfulness or confusion weakness ... cyclophosphamide (a medication that decreases the immune system�s response to autoimmune diseases), are generally used. In some ...
We have studied complement activation both in plasma samples and in lesional skin from patients with leukocytoclastic cutaneous vasculitis (LCV). Enzyme immunoassay (EIA) quantification of the complement activation markers, C3d,g and the terminal complement complex (TCC) in plasma, showed that their levels were significantly increased in 66% and 55% of the patients, respectively (n = 29) compared with healthy controls, whereas the standard measurements of C3, factor B, C1q, C4 and C2 were generally within normal range. Elevations of C3d,g and TCC levels in plasma were significantly correlated. Importantly, a significant correlation was found between the severity of the vasculitis and both C3d,g and TCC plasma levels. Immunofluorescence studies of skin biopsy specimens demonstrated simultaneous presence of perivascular dermal deposits of C3d,g and TCC in lesional skin from 96% and 80% respectively of the patients (n = 25). There was a significant correlation between the intensity of the deposits of both markers. Clusterin, a TCC inhibitory protein, was always found at the same sites of perivascular TCC deposits. Immunofluorescence studies at the epidermal basement membrane zone (BMZ) revealed in each case deposits of C3d,g which were accompanied by TCC deposits in 52% of the biopsy specimens. These data demonstrate that there is a local and systemic activation of the whole complement cascade in human LCV. The presence of both C3d,g and clusterin-associated TCC perivascular deposits suggests an intervention of a regulatory mechanism of local complement activation in LCV. Finally, measurement of plasma C3d,g and TCC appears to be a sensitive indicator of systemic complement activation and disease severity in LCV. Images Fig. 1 Fig. 2 PMID:8485913
Dauchel, H; Joly, P; Delpech, A; Thomine, E; Sauger, F; Le Loet, X; Lauret, P; Tron, F; Fontaine, M; Ripoche, J
Increasing evidence suggested that Toll-like receptors (TLRs) were critically involved in immune responses of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The current study aimed to investigate the expression of TLR-2, TLR-4 and TLR-9 in kidneys of patients with ANCA-associated vasculitis. Renal biopsy specimens were collected from 24 patients with AAV. The expression of TLR-2, TLR-4 and TLR-9 in kidneys was detected by immunohistochemistry. Double immunofluorescence staining was performed to detect the expression of TLRs on various kinds of cells. In renal specimens, immunohistochemical examination revealed that expression of TLR-2 and TLR-4 could be detected in the glomeruli of AAV patients, while TLR-2 and TLR-4 were scarcely detected in the glomeruli of normal controls. Double immunofluorescence staining of TLR-2, TLR-4 and CD31 indicated that TLR-4 and TLR-2 were expressed on endothelial cells in the glomeruli. In the tubulointerstitial compartment, expression of TLR-2, TLR-4 and TLR-9 could be detected in both AAV patients and normal controls. The mean optical density of TLR-2 and TLR-4 in the tubulointerstitial compartment in AAV patients were significantly higher than that in normal controls. Among AAV patients, correlation analysis showed that the mean optical density of TLR-4 in the glomeruli correlated inversely with the initial serum creatinine, the proportion of total crescents and the proportion of cellular crescents in renal specimens (r?=?-0�419, P?=?0�041; r?=?-0�506, P?=?0�012; r?=?-0�505, P?=?0�012, respectively). The expression of TLR-2 and TLR-4 was dysregulated in kidneys of AAV patients. The expression of TLR-4 in glomeruli was associated with the severity of renal injury. PMID:24773611
Wang, H; Gou, S-J; Zhao, M-H; Chen, M
Objective To estimate the coverage provided by SNOMED CT for clinical research concepts represented by the items on case report forms (CRFs), as well as the semantic nature of those concepts relevant to post-coordination methods. Design Convenience samples from CRFs developed by rheumatologists conducting several longitudinal, observational studies of vasculitis were selected. A total of 17 CRFs were used as the basis of analysis for this study, from which a total set of 616 (unique) items were identified. Each unique data item was classified as either a clinical finding or procedure. The items were coded by the presence and nature of SNOMED CT coverage and classified into semantic types by 2 coders. Measurements Basic frequency analysis was conducted to determine levels of coverage provided by SNOMED CT. Estimates of coverage by various semantic characterizations were estimated. Results Most of the core clinical concepts (88%) from these clinical research data items were covered by SNOMED CT; however, far fewer of the concepts were fully covered (that is, where all aspects of the CRF item could be represented completely without post-coordination; 23%). In addition, a large majority of the concepts (83%) required post-coordination, either to clarify context (e.g., time) or to better capture complex clinical concepts (e.g., disease-related findings). For just over one third of the sampled CRF data items, both types of post-coordination were necessary to fully represent the meaning of the item. Conclusion SNOMED CT appears well-suited for representing a variety of clinical concepts, yet is less suited for representing the full amount of information collected on CRFs. PMID:16799121
Richesson, Rachel L.; Andrews, James E.; Krischer, Jeffrey P.
Similarities have been noted in the histologic patterns of lymphomatoid granulomatosis and Epstein-Barr virus associated lymphoproliferative disease involving the lung. Epstein-Barr virus has also been identified by polymerase chain reaction in most cases of lymphomatoid granulomatosis; however, the precise cellular localization of Epstein-Barr virus sequences has not been extensively studied. We analyzed 10 cases of lymphomatoid granulomatosis involving the lung by immunohistochemistry and combined immunohistochemistry with in situ hybridization for Epstein-Barr virus, CD20, and CD45RO. All cases were selected from the files of the Armed Forces Institute of Pathology and met the clinical and histologic criteria for the diagnosis of lymphomatoid granulomatosis, grades 1 through 3. In all 10 cases, immunohistochemistry showed that most of the cells--small to medium-sized lymphocytes--were T cells (CD45RO+); however, a much smaller population of medium-sized to large atypical cells were B cells (CD20+). In each case, combined immunohistochemistry and in situ hybridization confirmed the presence of Epstein-Barr virus sequences within B (CD20+) cells and the absence of Epstein-Barr within T-cells (CD45RO+). Polymerase chain reaction analysis for immunoglobulin heavy-chain gene rearrangement identified a monoclonal pattern in six of nine cases tested, whereas analysis for T-cell receptor gamma-chain gene rearrangements was negative in three cases tested. On the basis of these findings, we hypothesize that most cases of lymphomatoid granulomatosis involving the lung represent a proliferation of Epstein-Barr virus infected B-cells with a prominent T-cell reaction and vasculitis, distinguishing these cases from angiocentric "T-cell lymphomas" in other sites, such as the head and neck. PMID:8037289
Guinee, D; Jaffe, E; Kingma, D; Fishback, N; Wallberg, K; Krishnan, J; Frizzera, G; Travis, W; Koss, M
Large vessel vasculitis (LVV) is an often-reported cause of inflammation of unknown origin (IUO) in elderly people. The objective of this study was to describe the usefulness of fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) and contrast-enhanced CT in early diagnosis and treatment follow-up of patients with LVV presenting as elderly onset IUO. We retrospectively compared contrast-enhanced CT findings and FDG-PET/CT findings of the patients diagnosed with LVV and 11 controls; all subjects were 50�years of age or older. We evaluated maximum standardised uptake value (SUVmax) and PET score of the aortic wall for quantitative comparison of FDG-PET/CT findings. We measured the aortic wall thickness (W) and its ratio against the radius (W/R) for quantitative comparison of aortic wall thickening by contrast-enhanced CT. After steroid treatment, we compared these values with those pre-treatment. Of 124 patients who were hospitalised due to advanced age and IUO, 88 underwent FDG-PET/CT and contrast-enhanced CT. Abnormal findings were observed on images from 78 patients. The findings were indicative of LVV in 13 patients (10.5�%), of whom more than half had only non-specific symptoms. Patients with LVV had significantly higher aortic wall SUVmax (3.85 vs. 1.95), PET scores by FDG-PET/CT, and aortic wall thicknesses by contrast-enhanced CT (3.8 vs. 2.6�mm) than controls. Significant improvement in aortic wall thickening was evidenced by reduced PET scores and by contrast-enhanced CT findings in patients who were followed up after treatment. LVV is an important cause of IUO with non-specific symptoms in elderly patients. Imaging examination comprising contrast-enhanced CT and FDG-PET/CT is useful for early diagnosis and early treatment evaluation of LVV, allowing for amelioration of reversible aortic wall thickening. PMID:24643395
Muto, Go; Yamashita, Hiroyuki; Takahashi, Yuko; Miyata, Yoko; Morooka, Miyako; Minamimoto, Ryogo; Kubota, Kazuo; Kaneko, Hiroshi; Kano, Toshikazu; Mimori, Akio
Introduction Low testosterone levels in men are associated with fatigue, limited physical performance and reduced health-related quality of life (HRQOL); however, this relationship has never been assessed in patients with anti-neutrophil cytoplasmic antibodies (ANCA) -associated vasculitides (AAV). The aim of this study was to assess the prevalence of androgen deficiency and to investigate the role of testosterone in fatigue, limited physical condition and reduced HRQOL in men with AAV. Methods Male patients with AAV in remission were included in this study. Fatigue and HRQOL were assessed by the multi-dimensional fatigue inventory (MFI)-20 and RAND-36 questionnaires. Results Seventy male patients with a mean age of 59 years (SD 12) were included. Scores of almost all subscales of both questionnaires were significantly worse in patients compared to controls. Mean total testosterone and free testosterone levels were 13.8 nmol/L (SD 5.6) and 256 pmol/L (SD 102), respectively. Androgen deficiency (defined according to Endocrine Society Clinical Practice Guidelines) was present in 47% of patients. Scores in the subscales of general health perception, physical functioning and reduced activity were significantly worse in patients with androgen deficiency compared to patients with normal androgen levels. Testosterone and age were predictors for the RAND-36 physical component summary in multiple linear regression analysis. Testosterone, age, vasculitis damage index (VDI) and C-reactive protein (CRP) were associated with the MFI-20 subscale of general fatigue. Conclusions This study showed that androgen deficiency was present in a substantial number of patients with AAV. Testosterone was one of the predictors for physical functioning and fatigue. Testosterone may play a role in fatigue, reduced physical performance and HRQOL in male patients with AAV. PMID:24028544
Clinical aspects of primary vasculitis
Conclusion��Vasculitic syndromes have now been recognized for some two centuries, and still pose diagnostic and other problems for clinicians.\\u000a In general, vasculitic syndromes cause substantial morbidity and mortality. Descriptions of larger series of patients, classification\\u000a efforts, and improved histological techniques have helped in dealing with these diseases. However, we still have to rely on\\u000a associations of certain clinical characteristics and
C. A. Stegeman; C. G. M. Kallenberg
The �other� vasculitis syndromes and kidney involvement
There are a number of vasculitides that are not confined to a specific vessel size, do not have characteristic features, and\\/or\\u000a are not secondary to another disease. Most of these vasculitides are rare in childhood. Beh�et disease is representative of\\u000a this group as it involves vessels of any size on both the arterial and venous side. In addition to renal
Seza Ozen
Churg-Strauss syndrome (CSS) is a small-vessel vasculitis characterized by severe asthma, lung/tissues infiltrates, extravascular necrotizing granulomas, and eosinophilia. Cutaneous involvement is common but may not be highly suggestive. Two typical cases of CSS with cutaneous involvement are herein reported, both females, 37 and 54�years old, presenting with lower limbs palpable purpura and urticarial lesions of the neck, respectively. A comprehensive review of the literature showed that cutaneous manifestations occurred in 40-81% of CSS patients and were the presenting sign in 14% of the patients. Moreover, a total of 68 cases of CSS with a detailed description of the cutaneous lesions have been published. In the majority of these patients, skin lesions allowed for the histopathological diagnosis of CSS. The most common clinical features were papulo-nodules with the histological picture of extravascular Churg-Strauss granuloma followed by purpuric and/or necrotic lesions in the lower limbs corresponding to small-vessel vasculitis with eosinophils. Less common lesions included urticarial lesions and livedo reticularis. Therefore, a high index of suspicion on skin lesions and the proper lesion selection for histological examination may be very important for early diagnosis of CSS. Clinical-pathological correlation is essential, as both clinical and histological features are not pathognomonic. PMID:20949297
Bosco, Laura; Peroni, Anna; Schena, Donatella; Colato, Chiara; Girolomoni, Giampiero
Skin rash and arthritis a simplified appraisal of less common associations.
Skin and joint manifestations are part of the clinical spectrum of many disorders. Well-known associations include psoriatic arthritis and arthritis associated with autoimmune connective tissue diseases. This review focuses on less common associations where skin lesions can provide easily accessible and valuable diagnostic clues, and directly lead to the specific diagnosis or limit the list of possibilities. This may also affect health care resources as diagnostic tests are often low-specific, highly expensive and poorly available. This group of diseases can be divided into two subsets, based on the presence/absence of fever, and then further classified according to elementary skin lesions (macular, urticarial, maculo-papular, vesico-bullous, pustular, petechial and nodular). In most instances joint involvement occurs as peripheral migrating polyarthritis. Erythematosus macular or urticarial rashes occur in most febrile disorders such as monogenic autoinflammatory syndromes, Schnitzler's syndrome, Still's disease and rheumatic fever and afebrile diseases as urticarial vasculitis. Pustular rash may be observed in chronic recurrent multifocal osteomyelitis (CRMO) and pyogenic arthritis with pyoderma gangrenosum and acne (PAPA) syndrome (both febrile) as well as in Behcet's disease and Synovitis, acne, pustulosis, hyperostosis and osteitis syndrome (both non-febrile). Papular lesions are typical of secondary syphilis, sarcoidosis, interstitial granulomatous dermatitis, papular petechial of cutaneous small-vessel vasculitis and nodular lesions of polyarteritis nodosa and multicentric reticulohistiocytosis all of which are afebrile. Differential diagnosis includes infections and drug reactions which may mimic several of these conditions. To biopsy the right skin lesion at the right time it is essential to obtain relevant histological information. PMID:23980929
Cozzi, A; Doria, A; Gisondi, P; Girolomoni, G
Wells' Syndrome Associated with Churg-Strauss Syndrome
Churg-Strauss syndrome (CSS) is a multisystem granulomatous vasculitis that is characterized by peripheral eosinophilia and the infiltration of eosinophils into systemic organs. The skin lesions of CSS consist mainly of palpable purpura and nodules. Wells' syndrome (WS) is a rare inflammatory dermatosis that is associated with recurrent granulomatous dermatitis and eosinophilia. Since these two diseases are rare, any overlap between them is very unusual. Herein, we report a patient with CSS, who initially presented a skin eruption of erythematous urticarial-plaques, vesicles, and blisters. Upon biopsy, the histology of these plaques indicated eosinophilic infiltration and "flame figures" within the dermis, which was consistent with a diagnosis of WS. Although the association between WS and CSS that was observed in our patient may be purely coincidental, it could also suggest a common pathogenetic background of these two distinct diseases, as both share several many common features. PMID:22148019
Lee, Soo Hyun; Roh, Mi Ryung; Jee, Hyunjoong; Chung, Kee Yang
Urticaria affects 15% to 20% of the population once or more during a lifetime. Chronic urticaria is a frequent recurrent eruption over a period greater than 6 weeks; the cause remains a mystery in more than 75% of cases. Urticaria and angioedema may be produced by immunologic or nonimmunologic means. Urticarial vasculitis, contact urticaria, mastocytosis, physical urticarias, dermatographism, cholinergic urticaria, localized heat urticaria, cold urticaria, aquagenic urticaria, and vibratory angioedema all require specific evaluation and treatment. Chronic idiopathic urticaria is usually controlled by antihistamines; depending on the circadian rhythm of the eruption, sedative or nonsedative antihistamines are prescribed. Some patients will require a combination of H1 and H2 antagonists, or even parenteral corticosteroids. PMID:1970697
Burrall, B. A.; Halpern, G. M.; Huntley, A. C.
Use of imaging studies in the diagnosis of vasculitis.
Imaging studies are necessary to determine disease extension and disease activity in the small-vessel vasculitides. Computed tomography (CT) and magnetic resonance imaging (MRI) increase the number of pathologic findings compared with conventional radiography. MRI delineates mucosal inflammation and granulomas in the paranasal sinuses, whereas CT provides information about osseous lesions. CT is superior to MRI for the detection of pulmonary lesions. Radiograph angiography has been the gold standard for medium- and large-vessel vasculitides for decades. Echocardiography and MRI correspond well with conventional angiography to assess cardiac involvement in Kawasaki disease. MRI, CT, and CT angiography are alternative noninvasive techniques to delineate vasculitic lesions in polyarteritis nodosa, Takayasu's arteritis, and large-vessel giant cell arteritis. Duplex ultrasonography has the greatest resolution. It delineates typical artery wall swelling in temporal arteritis and Takayasu's arteritis. Positron emission tomography can assess inflammatory activity of large arteries. PMID:15134599
Schmidt, Wolfgang A
Intradural vasculitis and hemorrhage in full sibling Welsh springer spaniels.
Two, full sibling, Welsh springer spaniel presented at 8 and 18 mo of age with rapidly progressive ataxia, recumbency, and pyrexia. The spinal cord contained extensive subdural hemorrhage and, in 1 dog, suppurative and necrotizing arteritis in the dura. The findings suggest a familial form of canine juvenile polyarteritis syndrome. PMID:12650042
Caswell, Jeff L; Nykamp, Stephanie G
Intradural vasculitis and hemorrhage in full sibling Welsh springer spaniels
Two, full sibling, Welsh springer spaniels presented at 8 and 18 mo of age with rapidly progressive ataxia, recumbency, and pyrexia. The spinal cord contained extensive subdural hemorrhage and, in 1 dog, suppurative and necrotizing arteritis in the dura. The findings suggest a familial form of canine juvenile polyarteritis syndrome. PMID:12650042
Caswell, Jeff L.; Nykamp, Stephanie G.
Neutrophilic dermatoses have long been known to be associated with autoinmune systemic diseases. Recently, a small number of cases of a disorder distinct from Sweet syndrome or bullous lupus erythematosus (LE) have been described as specifically related to systemic LE under diverse terms, including nonbullous neutrophilic dermatosis, nonbullous neutrophilic LE, and Sweet-like neutrophilic dermatosis. We describe 7 patients that developed urticarial lesions in the context of a known or concurrently diagnosed autoimmune connective tissue disease. Of a total of 7 patients, 6 were afflicted by systemic LE and 1 by rheumatoid arthritis and secondary Sj�gren syndrome. Histological findings in all patients included an interstitial and perivascular neutrophilic infiltrate with leukocytoclasia, vacuolar alteration along the dermal-edidermal junction, and no vasculitis. Most patients had active systemic disease at the time of the cutaneous eruption. Skin lesions resolved rapidly after the administration of immunomodulating agents. In conclusion, we provide additional evidence of the existence of a recently defined nonbullous neutrophilic dermatosis in the context of autoimmune connective tissue diseases and propose the term autoimmunity-related neutrophilic dermatosis as an appropriate designation. Furthermore, we believe that this entity should prompt physicians to screen the presence of an active systemic disorder in afflicted patients. PMID:23518639
Saeb-Lima, Marcela; Charli-Joseph, Yann; Rodr�guez-Acosta, Elva Dalia; Dom�nguez-Cherit, Judith
Revisiting cutaneous adverse reactions to pemetrexed
Pemetrexed (Alimta�) is a multitargeted antifolate drug approved as a single agent or in combination with cisplatin for the treatment of a small number of malignancies including advanced and metastatic non-squamous non-small cell lung cancer (NSCLC), and malignant pleural mesothelioma. This review reports the recent peer-reviewed publications and original findings regarding cutaneous adverse reactions (CARs) to pemetrexed. Pemetrexed-related CARs are frequently reported under the unspecific term �skin rash�. However, more specific diseases were tentatively identified as alopecias, urticarial vasculitis, acute generalized exanthematous pustulosis, toxic epidermal necrolysis, radiation recall dermatitis and pityriasis lichenoides. Most of the skin reactions occur shortly after pemetrexed administration. As with methotrexate-related CARs, the cell cycle arrest in the S phase may be regarded as a direct and major cause of the cytotoxic pathobiology. An adverse immune reaction is unlikely. In conclusion, pemetrexed is responsible for CARs exhibiting a variety of clinical presentations. Their origin is likely attributed to direct cytotoxicity following the cell cycle arrest in the S phase and cell necrosis. PMID:22866124
Pierard-Franchimont, Claudine; Quatresooz, Pascale; Reginster, Marie-Annick; Pierard, Gerald E.
Circulating Angiopoietin-2 as a Biomarker in ANCA-Associated Vasculitis
The endothelial-specific Angiopoietin-Tie2 ligand-receptor system is an important regulator of endothelial activation. Binding of angiopoietin-2 (Ang-2) to Tie2 receptor renders the endothelial barrier responsive to pro-inflammatory cytokines. We previously showed that circulating Ang-2 correlated with disease severity in a small cohort of critically ill patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis. The current study reassessed Ang-2 as a biomarker of disease activity and relapse in AAV. Circulating Ang-2 was measured in 162 patients with severe AAV (BVAS/WG?3, with or without glomerulonephritis) in a clinical trial. Ang-2 levels during active AAV were compared to levels in the same patients during remission (BVAS/WG?=?0). Levels in clinical subsets of AAV were compared, and association with future disease course was assessed. Ang-2 levels were elevated in severe disease (median 3.0 ng/ml, interquartile range 1.9�4.4) compared to healthy controls (1.2, 0.9�1.5). However, they did not reliably decline with successful treatment (median 2.6 ng/ml, interquartile range 1.9�3.8, median change ?0.1). Ang-2 correlated weakly with BVAS/WG score (r?=?0.17), moderately with markers of systemic inflammation (r?=?0.25�0.41), and inversely with renal function (r?=??0.36). Levels were higher in patients with glomerulonephritis, but levels adjusted for renal dysfunction were no different in patients with or without glomerulonephritis. Levels were higher in patients with newly diagnosed AAV and lower in patients in whom treatment had recently been started. Ang-2 levels during active disease did not predict response to treatment, and Ang-2 levels in remission did not predict time to flare. Thus, Ang-2 appears to have limited practical value in AAV as a biomarker of disease activity at time of measurement or for predicting future activity. PMID:22279570
Monach, Paul A.; Kumpers, Philipp; Lukasz, Alexander; Tomasson, Gunnar; Specks, Ulrich; Stone, John H.; Cuthbertson, David; Krischer, Jeffrey; Carette, Simon; Ding, Linna; Hoffman, Gary S.; Ikle, David; Kallenberg, Cees G. M.; Khalidi, Nader A.; Langford, Carol A.; Seo, Philip; St. Clair, E. William; Spiera, Robert; Tchao, Nadia; Ytterberg, Steven R.; Haubitz, Marion; Merkel, Peter A.
Vasculitis reminds us that 'Ockham's razor' may still apply in the elderly.
A 76-year-old woman with a history of stage IV chronic kidney disease and hypertension was admitted to the hospital for progressive weakness and acute on chronic kidney injury. She was found to have anaemia requiring transfusion. On the evening of admission, she had worsening respiratory status with subsequent diagnosis of a non-ST-elevation myocardial infarction, pulmonary oedema on chest x-ray presumed to be a result of heart failure, and a possible transfusion reaction. A kidney biopsy performed as part of her ongoing nephrology evaluation revealed granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis). Subsequent bronchoalveolar lavage confirmed diffuse alveolar haemorrhage as the explanation for her anaemia and respiratory decline. She underwent immunosuppressive therapies and initiated dialysis, but subsequently chose hospice care and died 2 months after her diagnosis. PMID:23505087
Mount, Hillary R
Drug-eluting balloons (DEBs) coated with the antiproliferative agent paclitaxel may improve primary patency by reducing recurrent luminal stenosis. A proportion of the active drug and excipient coating are known to embolize distally, but until now, there have been no reports of adverse events resulting from their use. We report an unusual case of a painful nodular, biopsy specimen-proven vasculitic rash that afflicted the ipsilateral lower limb of a patient after superficial femoral artery treatment with a DEB. This adverse event may have implications for the use of DEB in this and other vascular territories. PMID:23642919
Thomas, Shannon D; McDonald, Robert R A; Varcoe, Ramon L
Large vessel vasculitis without temporal artery involvement: Isolated form of giant cell arteritis?
Diffuse arterial involvement in giant cell arteritis (GCA) is well recognized. By contrast, GCA clinically isolated to large vessels without cephalic, rheumatologic or systemic symptoms represents a much rarer manifestation of the disease.
M. Lambert; A. Weber; B. Boland; J. F. Plaen; J. Donckier
Recent Understanding on Diagnosis and Management of Central Nervous System Vasculitis in Children
Central nervous system vasculitides in children may develop as a primary condition or secondary to an underlying systemic disease. Many vasculitides affect both adults and children, while some others occur almost exclusively in childhood. Patients usually present with systemic symptoms with single or multiorgan dysfunction. The involvement of central nervous system in childhood is not frequent and it occurs more often as a feature of subtypes like childhood polyarteritis nodosa, Kawasaki disease, Henoch Sch�nlein purpura, and Bechet disease. Primary angiitis of the central nervous system of childhood is a reversible cause of severe neurological impairment, including acute ischemic stroke, intractable seizures, and cognitive decline. The first line therapy of CNS vasculitides is mainly based on corticosteroids and immunosuppressor drugs. Other strategies include plasmapheresis, immunoglobulins, and biologic drugs. This paper discusses on current understanding of most frequent primary and secondary central nervous system vasculitides in children including a tailored-diagnostic approach and new evidence regarding treatment. PMID:23008735
Iannetti, Ludovico; Zito, Roberta; Bruschi, Simone; Papetti, Laura; Ulgiati, Fiorenza; Nicita, Francesco; Del Balzo, Francesca; Spalice, Alberto
Non-necrotizing Granulomatous Pulmonary Vasculitis Mimicking Lung Cancer on PET/CT
Fluorodeoxyglucose positron emission tomography (18FDG-PET) scan has become a valuable resource in the staging of lung cancer. Inflammation is known to cause false positives on 18FDG-PET scan. In the absence of symptoms suggesting a diagnosis of an inflammatory condition, 18FDG-avid lung masses on PET/CT scan is strongly suggestive of a diagnosis of lung cancer, rather than an inflammatory condition. We report the case of a 57-year-old man, with a history of heavy smoking and working in the sandblasting industry, with two suspicious 18FDG-avid nodules in the left lung. Surgical specimens of these nodules revealed findings suspecting giant cell arteritis rather than malignancy. PMID:25360406
Dixon, Matthew; Shaw, Jason; Rankin, Linda; Lazzaro, Richard
right eye since childhood, and had had peripheral retinal vascular occlusion. Although the exact of night blindness, characterized by diffuse yellow spots and flecks in the fundus and by delayed dark adaptation.1 It is generally classed as one of the night-blinding disorders because the typical patient shows
Palczewski, Krzysztof
In vivo approaches to investigate ANCA-associated vasculitis: lessons and limitations
Anti-neutrophil cytoplasm autoantibody (ANCA)-associated diseases are autoimmune conditions characterized by necrotizing inflammation\\u000a of small blood vessels. The immunogenesis and etiology of these conditions are unknown, but our knowledge of the immunopathogenesis\\u000a has increased considerably in recent years. In this review, we discuss the animal models currently used to investigate the\\u000a mechanisms of vascular injury and to test novel therapies. We
Peter Heeringa; Mark A Little
Therapy Insight: the recognition and treatment of retinal manifestations of systemic vasculitis
A variety of retinal signs can occur in patients who have systemic vasculitides, or who experience complications of these diseases or their treatment. Although treatment of these retinal manifestations is usually the treatment of the systemic disease, specific treatment is occasionally indicated to preserve vision. The more prevalent of the systemic vasculitides are giant cell arteritis, polyarteritis nodosa, Wegener's granulomatosis,
Petros Aristodemou; Miles Stanford
Increased Fibroblast Elastase Activity in Acquired Cutis laxa
Background: Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity. Observation: We report a 21-year-old woman, who presented with acquired cutis laxa on the face and the ear lobes. Urticarial papules had preceded for 6 years. There was no systemic involvement. Skin specimens were obtained from lax skin and urticarial papules, and
A. Bouloc; G. Godeau; J. Zeller; J. Wechsler; J. Revuz; A. Cosnes
autoimmune diseases (rheumatoid arthritis, Wegener granulomatosis, Churg-Strauss syndrome/allergic granulomatosis), inflammatory/granulomatous disorders (sarcoidosis, inflammatory bowel disease), and infections (chronic hepatitis,
Gaertner, Erich M; Switlyk, Stephen A
The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired\\/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and\\/or bone pain, enlarged lymph nodes,
Dan Lipsker
Introduction We report a rare case of fulminant vasculitic mononeuropathy resulting in brachial diplegia, with suspected brainstem and autonomic nervous system involvement in a patient with diabetes mellitus. Case presentation A 58-year-old Hispanic Caucasian man with diabetes mellitus presented with a 1-year history of progressive bilateral upper extremity weakness, orthostatic intolerance and progressive memory decline. Diagnostic evaluation including laboratory tests for progressive encephalopathies, systemic inflammatory and non-inflammatory neuropathies, cerebrospinal fluid analyses, electrodiagnostic studies, and nerve biopsy were performed. Clinical examination revealed moderate cognitive deficits on the Montreal Cognitive Assessment scale, bilateral facial weakness and weakness of bilateral shoulder girdle and intrinsic hand muscles. Cerebrospinal fluid analyses revealed elevated protein and an elevated immunoglobulin G synthesis rate, suggesting an immune-mediated process. Further laboratory work up was non-diagnostic. Electrodiagnostic studies demonstrated chronic asymmetric axonal mononeuropathies with ongoing denervation. A superficial radial nerve biopsy showed a chronic vasculitic neuropathy. Glucocorticosteroid treatment, symptomatic pharmacologic and supportive non-pharmacologic therapies resulted in improved clinical outcomes despite challenges with glycemic control. Conclusions This case report emphasizes the importance of a thorough evaluation of atypical or uncommon neuromuscular presentations in diabetic patients without etiological presumptions. This is necessary in order to promptly establish a diagnosis, initiate appropriate therapies and prevent irreversible nerve injury. PMID:24083508
months. Both regimens are preceded by IV methyl- prednisolone of between 1 and 3 grams. Oral GC may be provided as prednisolone or prednisone according to local practice. Table 1 Glucocorticoid dosing in the standard and reduced-dose groups of PEXIVAS... in rheumatoid arthritis: an analysis of serious adverse events. Am J Med 1994, 96:115�123. 23. Wolfe F, Caplan L, Michaud K: Treatment for rheumatoid arthritis and the risk of hospitalization for pneumonia: associations with prednisone, disease...
Walsh, Michael; Merkel, Peter A; Peh, Chen Au; Szpirt, Wladimir; Guillevin, Lo�c; Pusey, Charles D; deZoysa, Janak; Ives, Natalie; Clark, William F; Quillen, Karen; Winters, Jeffrey L; Wheatley, Keith; Jayne, David; on behalf of the PEXIVAS Investigators
Pharmacological studies on nonimmunologic contact urticaria in guinea pigs
In the present study we examined the effects of chlorpheniramine and ranitidine, indomethacin, BW755C (an inhibitor of cyclo-oxygenase and lipooxygenase enzymes of arachidonic acid metabolism), dexamethasone, and capsaicin on nonimmunologic contact urticaria (NICU) induced in the guinea pig ear by benzoic acid, cinnamic acid, cinnamic aldehyde, methyl nicotinate, diethyl fumarate, or dimethyl sulfoxide. The intensity of edema in the urticarial
A. Lahti; D. M. McDonald; R. Tammi; H. I. Maibach
An elderly male noticed urticarial patches corresponding to cutaneous B cell lymphoma lesions after rituximab treatment. Along with the resolution of urticaria, the lymphoma lesions completely remitted without recurrence. In this communication, we present an interesting case and the pathophysiological findings of a wheal attack in a case with tumor remission following systemic treatment with rituximab, a monoclonal anti-CD20 antibody.
Itoi, Saori; Tanemura, Atsushi; Hayashi, Misa; Nagata, Naoko; Tani, Mamori; Katayama, Ichiro
Systemic Immediate Allergic Reactions to Arthropod Stings and Bites
Most of the encounters with biting and stinging insects result in more or less pronounced localized reactions. Typically, urticarial wheals and papular reactions are observed. Less often local bullous or hemorrhagic or disseminated papular reactions, particularly in children and immunologically naive adults, may be seen. With the exception of bee and wasp venom allergies, immediate-type allergic reactions to arthropod stings
Andreas J. Bircher
Palpable purpura and uveitis precipitated by splenectomy in an atypical case of Cogan's syndrome.
A patient with a baffling multisystem illness developed uveitis and palpable purpura shortly after a splenectomy, leading to the diagnosis of vasculitis. Clearance by the mononuclear phagocyte system is a key factor determining the pathogenicity of circulating immune complexes, which are known to be the cause of some forms of vasculitis. The sudden appearance of uveitis and cutaneous vasculitis in an illness of many years' duration was probably a direct result of removal of the spleen. PMID:1920316
Wohlgethan, J R; Stilmant, M M; Smith, H R
Genetics Home Reference: Complement component 2 deficiency
... also associated with an increased risk of developing autoimmune disorders such as systemic lupus erythematosus (SLE) or vasculitis. Autoimmune disorders occur when the immune system malfunctions and attacks ...
Diagnostic dilemma in autoinflammatory disease in two patients: does the name matter?
The systemic autoinflammatory diseases are inflammatory disorders characterized by uncontrolled inflammation of the innate immune system. A common monogenic autoinflammatory disease is familial Mediterranean fever (FMF), associated with mutations in the MEFV gene. Another autoinflammatory disease group is cryopyrin-associated periodic syndromes (CAPS), which are characterized by urticarial rash and mutations of the gene NLRP. Systemiconset juvenile idiopathic arthritis (soJIA) is classified as a multifactorial autoinflammatory disease. We report two cases of systemic autoinflammatory disease with homozygous E148Q mutation in the FMF gene. They had unusual features, such as urticarial rash, non-erysipeloid erythema, lymphadenopathy, and hepatosplenomegaly, and neurological findings in one. These patients met the "definition" criteria for FMF with two mutations in the MEFV gene. They fit the "description" criteria for CAPS with their fever, urticaria, and other clinical features. They also met the "classification" criteria for soJIA, with the fever, rash, arthritis, and accompanying systemic features. PMID:24217079
G�lhan, Bora; B�y�kcam, Ay?e; Touitou, Isabelle; �zen, Seza
Drug-Eluting Coronary Stent Allergy: A New and Unique Patient Safety Issue
Case Presentation: A 56-year-old woman underwent placement of a sirolimus-eluting Cypher stent for treatment of unstable angina. One day after stent placement, an urticarial rash and pruritus developed on the right groin at the catheter insertion site. She was started on a histamine antagonist for symptom relief. Soon after discharge, diffuse urticaria and pruritus developed. Her symptoms persisted despite treatment
Edgar A. Martorell; Juan Guarderas; Jorge Trejo; Kenneth Calamia
Dermatitis herpetiformis (DH) is a relatively rare skin disorder with an estimated incidence of 1:10000 in the UK. It is characterized by urticarial plaques and blisters on the elbows, buttocks, and knees, although other sites may also be involved. The eruption tends to be persistent: only 10�15% of patients have spontaneous remission over a 25-year study period. The disease is
Lionel Fry
Vasculitides in hairy cell leukemia
Forty-two cases of vasculitis coincident with hairy cell leukemia (HCL) havebeen reported, of which 17 had panarteritis nodosa (PAN), 21 had cutaneous leukocytoclastic vasculitis (LCV), and 4 had vessel wall infiltration by hairy cells. PAN generally occurred after the diagnosis of HCL, splenectomy, and infection. HBs antigen was detected in 3 of 12 patients tested, whereas immune complexes were positive
Paul Hasler; Hansj�rg Kistler; Heini Gerber
Sweet's syndrome: histological and immunohistochemical study of 15 cases
Conventional histology and immunoperoxidase staining for fibrin, immunoglobulins, and complement components were used to look for evidence of cutaneous vasculitis and immune complex deposition in Sweet's syndrome. These features were not identified in any of the 15 cases studied. The lack of any vasculitis emphasises the distinctive character of Sweet's syndrome when compared with certain spontaneous and experimentally induced inflammatory
J J Going; S M Going; G W Beveridge
Mixed cryoglobulinemia: demographic, clinical, and serologic features and survival in 231 patients
BackgroundMixed cryoglobulinemia (MC) is a systemic vasculitis secondary to circulating immune complex deposition in the small vessels. In the overwhelming majority of patients, hepatitis C virus (HCV) infection represents the triggering factor of the disease. MC is characterized by multiple organ involvement, mainly skin, liver, renal, peripheral nerves, and less frequently by widespread vasculitis and cancer.
Clodoveo Ferri; Marco Sebastiani; Dilia Giuggioli; Massimiliano Cazzato; Giovanni Longombardo; Alessandro Antonelli; Rodolfo Puccini; Claudio Michelassi; Anna Linda Zignego
Hairy cell leukemia presenting initially with symptoms of Beh�et's disease.
Vasculitis is relatively uncommon in lymphoproliferative disease and may predate the diagnosis of lymphoproliferative disease. Many vasculitides have been associated with hairy cell leukemia (HCL), including polyarteritis nodosa (PAN) and leukocytoclastic vasculitis. We herein report a case whose initial presentation was like Beh�et's disease (BD) (arthritis, oral and genital ulcerations, papulopustular skin lesions) in addition to pancytopenia, but turned out to have HCL. Because of the overlap between their symptoms, like oral ulcerations, skin lesions, arthritis and constitutional findings, HCL and BD may mimic each other. We should keep in mind other reasons for vasculitis such as lymphoproliferative disease, especially whose who have hematological abnormalities such as pancytopenia. PMID:24762098
Oksuz, Mustafa Ferhat; Coskun, Belkis Nihan; Tufan, Ayse Nur; Orucoglu, Nurdan; Dalkilic, Ediz; Ozt�rk Nazl?o?lu, H�lya; Pehlivan, Yavuz
... polymyalgia rheumatica (also called PMR). What is giant cell arteritis? GCA is a type of vasculitis or ... right away to their doctors. What causes giant cell arteritis? As with PMR, the cause of GCA ...
Histological findings after brown recluse spider envenomation.
Histologic specimens from 41 rabbits were studied for changes resulting from the manual injection of brown recluse spider venom. Major findings included a mixed inflammatory cell infiltrate, coagulative tissue necrosis, and vasculitis. All specimens demonstrated a well-delineated zone of eosinophilic staining recognizable as "mummified" coagulative necrosis of the epidermis and dermis. A dense band of neutrophils bordered the zone of necrosis. Immediately adjacent to the neutrophilic band, small vessel vasculitis was a universal finding. Degranulated eosinophils and neutrophils and macrophages filled with eosinophilic granules were common. Inflammatory foci were often centered on groups of lipocytes within the dermis. Large vessel vasculitis resembling that seen in polyarteritis nodosa was present deep to 7 of the 40 eschars. Large vessel vasculitis may contribute to the large zones of necrosis seen after some brown recluse spider bites. Eosinophils may play a role in tissue damage after envenomation. PMID:10871067
Elston, D M; Eggers, J S; Schmidt, W E; Storrow, A B; Doe, R H; McGlasson, D; Fischer, J R
POLYNEUROPATHY + NEURONOPATHY: Points in Differential Diagnosis 1. Selective Functional Involvement
; Horner's Treatable neuropathies Diabetes; Vasculitis; CIDP; Lymphoma; Infection; Toxic; Compression Motor Motor Neuropathy: Multifocal (MMN); Distal Motor Neuron Disorders Lower Motor Neuron (LMN): BVv Toxic: Pb; Dapsone; Botulism; Tick Acute: Porphyria; Axonal GBS (AMAN); Polio; West Nile Diabetic
Baloh, Bob
A Real World, Observational Registry of Chronic Wounds and Ulcers
Diabetic Foot; Varicose Ulcer; Pressure Ulcer; Surgical Wound Dehiscence; Vasculitis; Skin Ulcer; Leg Ulcer; Wounds and Injuries; Pyoderma; Peripheral Arterial Disease; Diabetic Neuropathies; Lymphedema; Venous Insufficiency; Diabetes Complications; Amputation Stump
Sequential strokes in a hyperacute stroke unit
Vasculitis is a rare, but treatable condition that can present to hyperacute stroke units. Thrombolysis does not treat the underlying pathology, and a rapidly evolving clinical picture drives clinical decision often before all the investigation results are available.
Ganesalingam, Jeban; Buddha, Sandeep; Carlton-Jones, Anoma L
Genetics Home Reference: Granulomatosis with polyangiitis
... granulomas grow, they can invade surrounding areas, causing tissue damage. The signs and symptoms of GPA vary based ... kidneys are commonly affected in people with GPA. Tissue damage caused by vasculitis in the kidneys can lead ...
Spontaneous ureteral rupture in a patient with systemic lupus erythematosus
A patient with known systemic lupus erythematosus had fever and symptoms of a lower urinary tract infection. Bone scintigraphy showed left ureteral perforation and necrosis with no demonstrable nephrolithiasis. It is speculated that this episode was due to lupus vasculitis.
Benson, C.H.; Pennebaker, J.B.; Harisdangkul, V.; Songcharoen, S.
... results will open in a new tab or window. Search MedlinePlus : Participation in Clinical Trials is one way you can help advance research into finding better treatments and perhaps even a cure for vasculitis! VCRC- ...
... results will open in a new tab or window. Search MedlinePlus : Participation in Clinical Trials is one way you can help advance research into finding better treatments and perhaps even a cure for vasculitis! VCRC- ...
... Specific forms of vasculitis treated with MTX include Wegener�s granuloma t o s i s , Microscopic polyangiitis, Churg-Strauss ... 000/mm3. Do I need to take an antibiotic? Possibly. Depending on how much medication you take ...
Histology Self-Study Presentation Ellen O'Dea
. Systemic lupus erythematosus (SLE) 3. Scleroderma (Systemic sclerosis) 4. Crystal induced disease (Gout, Autoimmune disease Division of Rheumatology, Allergy,and Immunology Main diseases: 1. Rheumatoid arthritis 2: Rheumatoid Arthritis Systemic Lupus Erythematosus Sclerosis Gout Vasculitis #12;Lymphoid System Cells
Gleeson, Joseph G.
38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.
Code of Federal Regulations, 2010 CFR
...coagulation with renal cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate as renal dysfunction....
38 CFR 4.115b - Ratings of the genitourinary system-diagnoses.
Code of Federal Regulations, 2011 CFR
...7540Disseminated intravascular coagulation with renal cortical necrosis: Rate as renal dysfunction. 7541Renal involvement in diabetes mellitus, sickle cell anemia, systemic lupus erythematosus, vasculitis, or other systemic disease processes. Rate...
... forms of vasculitis that do not respond to steroids ( prednisone ) alone, cyclophosphamide may be a life�saving medicine. ... a fatal illness, even with high doses of steroids. Now, with the combination of cyclophosphamide and prednisone, ...
A 31-year-old woman with Cogan syndrome (a rare form of systemic vasculitis) was evaluated for a cold, painful left foot with diminished pulses. Arteriography demonstrated thrombosis of the left popliteal artery with evidence of vasculitis. Thrombolytic therapy was begun with initial success but eventual rethrombosis. After reinitiating thrombolytic therapy combined with intraarterial vasodilator therapy, successful angioplasty was performed with sustained results, at 6-month follow-up.
Bastug, Demir E.; Dominic, Anthony; Ortiz, Orlando [Robert C. Byrd Health Sciences Center, Department of Radiology, West Virginia University (United States); DiBartolomeo, Anthony G. [Robert C. Byrd Health Sciences Center, Department of Medicine, Rheumatology Section, West Virginia University (United States); Kotzan, Jeffrey M. [Robert C. Byrd Health Sciences Center, Department of Radiology, West Virginia University (United States); Abraham, F. Matthew [Robert C. Byrd Health Sciences Center, Department of Medicine, West Virginia University (United States)
Facial heliotrope rash as the initial manifestation of acute myelomonocytic leukemia.
The association of leukocytoclastic vasculitis or dermatomyositis with malignancies has been reported. We describe a patient who developed a skin rash, histologically compatible with dermatomyositis, which during the course of the disease switched to leukocytoclastic vasculitis, which was accompanied with peripheral blood pancytopenia in the absence of any specific pathological manifestation from the bone marrow three years prior to the diagnosis of acute myelomonocytic leukemia (AMML). PMID:9168450
Tzavara, V; Stamoulis, K; Aroni, K G; Kordossis, T; Boki, K A
The significance of cell-bound antigen-specific IgE in chronic urticaria.
Radioallergosorbent test (RAST) was performed on the sera obtained from chronic urticarial patients from whose leucocytes significant histamine release by mite antigen was observed. In 14 out of 19 cases investigated, no mite-antigen-specific IgE was detected in their sera by RAST, in spite of significant mite-antigen-induced histamine release from their leucocytes. The results suggest that type I reaction may be involved in chronic urticaria and the cell-bound, antigen-specific IgE may be important rather than the circulating antigen-specific IgE in this disease. PMID:6162407
Yamamoto, S; Numata, T; Yamura, T
Contact urticaria from cinnamic aldehyde.
Lip swelling developed following the use of a cinnamic aldehyde-containing mouthwash in a woman with allergic rhinitis and asthma. Open application of the mouthwash on the antecubital fossae of the patient and control subjects produced immediate erythematous or urticarial responses in all tested individuals. Subsequent investigation established cinnamic aldehyde as the responsible ingredient. The incidence of positive reactions was shown to depend on site of application, vehicle, concentration, and time of reading. Of several related cinnamates, only cinnamic acid produced easily detectable responses at similar concentrations. PMID:7352767
Mathias, C G; Chappler, R R; Maibach, H I
A case of palpable purpura and nephropathy: Occam's Razor or Hickam's Dictum.
Vasculitis causing palpable purpura, nephropathy, and hematologic abnormalities is a well-known entity. However, sometimes, vasculitis may not be the primary cause but is part of a systemic disease. Literature suggests that infections like HIV can induce nephropathy and antineutrophilic cytoplasmic antibody-positive vasculitis, which is different from the well-known entity of "antineutrophilic cytoplasmic antibody-associated vasculitis." We present a 46-year-old female patient with a history of intravenous drug abuse who reported with a rash, swelling, and palpable purpura of the lower extremities. Peripheral smear showed no evidence of disseminated intravascular coagulation or thrombotic thrombocytopenic purpura; metabolic profile showed acute kidney injury. She was found to be HIV- and hepatitis C-positive. Immunologic workup was positive for both MPO and PR3 antineutrophilic cytoplasmic antibodies and negative for cryoglobulins; complement levels were low. Skin biopsy showed leukocytoclastic vasculitis but kidney biopsy was negative for any immunologic involvement; it showed only glomerulosclerosis. Thus, it was thought that nephropathy and vasculitis, in this case, are two distinct pathologic processes, both induced by infection (HIV and/or hepatitis C). The patient responded to low-dose steroid therapy. She was later started on the definitive therapy, the highly active antiretroviral therapy regimen. This case illustrates the fact that low-dose steroids can still be a good alternative in acute situations in patients at risk from immunosuppression. PMID:21248615
Mandhadi, Ranadeep; Kodumuri, Vamsi; Arora, Rohit; Puneet Singh, Param; Adigopula, Shashi; Chua, Serafin
Bilateral renal ischemia after kyphoplasty and clodronate treatment: a case report
Introduction The most common adverse effects associated with bisphosphonates are renal toxicity, acute-phase reactions, gastrointestinal toxicity, osteonecrosis of the jaw, transitory fever and uveitis. We report a unique adverse case of vasculitis induced by clodronate. Case presentation A 61-year-old Caucasian woman developed bilateral renal ischemia after kyphoplasty and clodronate treatment for lumbar vertebral fracture. Tests revealed a vasculitis due to clodronate treatment. The antithrombotic and immunosuppressive drugs allowed us to reduce the extent of the renal ischemia. In the following months the increased auto-antibodies returned to the healthy physiological range, but the chronic renal failure persisted. Conclusions Drug-induced vasculitis is an inflammation of blood vessels caused by the use of various pharmaceutical agents. The spectrum of drug-induced vasculitis can range from cutaneous rashes to fatal multi-organ involvement. To the best of our knowledge this is the first documented case of drug-induced vasculitis caused by clodronate in the literature. Previously, it was verified that clodronate injection could increase the pro-apoptotic action on immune cells. Further studies are necessary to clarify the role of bisphosphonates on drug-inducing vasculitis. PMID:24571672
Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener's granulomatosis).
Granulomatosis with polyangiitis (GPA, formerly known as Wegener's Granulomatosis) is an autoimmune small vessel vasculitis which is highly associated with anti-neutrophil cytoplasmic antibodies (ANCA). The hallmarks of this condition are systemic necrotising vasculitis, necrotising granulomatous inflammation, and necrotising glomerulonephritis. The aetiology of granulomatosis with polyangiitis is linked to environmental and infectious triggers inciting onset of disease in genetically predisposed individuals. Anti-neutrophil cytoplasmic antibodies are pathogenic and play an important role in the pathogenesis of this disease, although ANCA positivity is not essential for a clinical diagnosis of granulomatosis with polyangiitis. Granulomatosis with polyangiitis is diagnosed based on clinical manifestations of systemic vasculitis and histological evidence of necrotising vasculitis or granulomatous inflammation. This small vessel vasculitis may present as limited disease of the ears, nose and upper airways or mild, moderate or severe systemic disease. Immunosuppression and adjuvant therapies have contributed to the improved prognosis of granulomatosis with polyangiitis over the past decades. Treatment strategies are tailored to the severity of the disease. They are based on published evidence of the efficacy and safety of the immunosuppressive drugs indicated to manage active vasculitis and maintain clinical remission. This review will summarise the history, aetiology, pathogenesis, classification, diagnosis and management of granulomatosis with polyangiitis. PMID:24485158
Lutalo, Pamela M K; D'Cruz, David P
Measurement of anti-IgA antibodies by a two-site immunoradiometric assay
To enable the detection of IgG class, anti-IgA antibodies and to investigate the possible occurrence of IgE class, anti-IgA antibodies, we developed a solid phase immunoradiometric assay, which uses purified IgA coupled covalently to microcrystalline cellulose as an immunosorbent. Radiolabeled, Fc specific anti-IgG and anti-IgE antibodies were used to detect specific aIgA after incubation of test sera or controls with the immunosorbent. IgG-aIgA were detected by the IRA in 100 and 67% of control sera with class specific and limited specificity aIgA. The IRA was sensitive to approximately two ng of class specific IgG-aIgA. IgG-aIgA also were detected by IRA in 7.9% of sera from patients with urticarial transfusion reactions and 73% of sera from patients with ataxia telangiectasia and IgA deficiency. Sera from 50 normal blood donors did not have detectable IgG-aIgA. Tests for IgE-aIgA were negative in all cases, including control sera with class specific IgG-aIgA. We conclude that the IRA is a sensitive and reproducible method for detection of class specific and limited specificity IgG-aIgA, and that IgE-aIgA do not mediate urticarial transfusion reactions.
Homburger, H.A.; Smith, J.R.; Jacob, G.L.; Laschinger, C.; Naylor, D.H.; Pineda, A.A.
The Schnitzler syndrome is a rare and underdiagnosed entity which is considered today as being a paradigm of an acquired/late onset auto-inflammatory disease. It associates a chronic urticarial skin rash, corresponding from the clinico-pathological viewpoint to a neutrophilic urticarial dermatosis, a monoclonal IgM component and at least 2 of the following signs: fever, joint and/or bone pain, enlarged lymph nodes, spleen and/or liver, increased ESR, increased neutrophil count, abnormal bone imaging findings. It is a chronic disease with only one known case of spontaneous remission. Except of the severe alteration of quality of life related mainly to the rash, fever and pain, complications include severe inflammatory anemia and AA amyloidosis. About 20% of patients will develop a lymphoproliferative disorder, mainly Waldenstr�m disease and lymphoma, a percentage close to other patients with IgM MGUS. It was exceedingly difficult to treat patients with this syndrome until the IL-1 receptor antagonist anakinra became available. Anakinra allows a complete control of all signs within hours after the first injection, but patients need continuous treatment with daily injections. In many aspects, the Schnitzler syndrome resembles the genetically determined auto-inflammatory syndromes involving activating mutations of the NLRP3 inflammasome. This latter point and its consequences will be addressed. PMID:21143856
Hypertensive encephalopathy in childhood. Diagnostic problems.
Oppenheimer and Fischberg's vasoconstriction-hypothesis on the pathogenesis of hypertensive encephalopathy was subsequently supported by animal experiments. Later on the role of decompensation of the autoregulatory mechanism of the cerebral blood flow was revealed. The transient symptomatology comprises headache, seizures, focal cerebral symptoms (hemiplegia etc.), visual disturbances, mental disorders, papiledema etc. The age-dependency of the influence of edema is probably expressed by the predominance of seizures in childhood and the long duration of the symptoms in our third and fourth patient. The differentiation between hypertensive encephalopathy and a local complication of hypertension (hemorrhage) can be difficult, not at least because the first disturbance may be followed by the second (patient 3). Hypertension is not always present as initial symptom (patient 1 and 2). Hence a series of blood pressure readings is required in acute cerebral incidents in childhood. Steroid-treatment may lead, especially in patients suffering from a hypocomplementemic form of membranoproliferative glomerulonephritis, to a sudden rise of the blood pressure and subsequently to hypertensive encephalopathy (patients 2 and 3). Hypertensive encephalopathy is a neuropediatric emergency. The urgent treatment with dioxaside, fursemide and sodium nitroprusside is shortly reviewed. PMID:988919
van Vught, A J; Troost, J; Willemse, J
Current concept and epidemiology of systemic vasculitides.
Although a new classification algorithm for systemic vasculitides was proposed by Watts et al. and the Chapel Hill Consensus Conference (CHCC) was updated in 2012, there are currently no validated diagnostic criteria for systemic vasculitides. The Diagnostic and Classification Criteria for Vasculitis study (DCVAS) is a global study to develop and improve the diagnostic criteria for systemic vasculitides. The epidemiology of systemic vasculitides differs widely among countries. For example, in the case of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, patients with microscopic polyangiitis (MPA) and with positivity for MPO-ANCA are predominant in Asian countries, whereas patients with granulomatosis with polyangiitis (GPA) and with positivity for PR3-ANCA are predominant in northern Europe and the United States. Interstitial lung disease (ILD) occurs more frequently in Asian patients compared with patients in Europe. The incidence and the prevalence of large-vessel vasculitis also differ significantly. Giant cell arteritis (GCA) occurs frequently in northern Europe, unlike Takayasu arteritis (TAK). The ethnic and regional differences in the incidence, prevalence and clinical characteristics of patients with vasculitis should be recognized when we diagnose and treat patients with vasculitis using criteria, and should also be considered when interpreting the results from clinical studies. PMID:25339434
Katsuyama, Takayuki; Sada, Ken-Ei; Makino, Hirofumi
An 11-year and 10-month-old girl with purpura and chest pain.
Mucosa-associated lymphoid tissue lymphoma (MALToma) is a type of B-cell lymphoma. Case reports of childhood thymic MALToma and its association with vasculitis are rarely found in the related literature. Herein, we present a report of an 11-year and 10-month-old girl who was initially diagnosed with cutaneous vasculitis characterized by nonthrombocytopenic palpable purpura, positive antinuclear antibody and anti-SSA (Ro) antibody. Eight months later, a thymic mediastinal mass was found. Surgical excision was performed and results of pathological analysis revealed an extranodal marginal zone CD20(+) B-cell MALToma. Benign response to the chemotherapeutic regimen of Berlin-Frankfurt-M�nster group NHL-BFM 90 R2 without relapse was noted in 2 years of follow-up. For the first time, our case demonstrated some clinical evidence of the association between vasculitis and childhood MALToma. PMID:23006506
Chen, Pei-Hsuan; Chiang, Bor-Luen; Lu, Meng-Yao; Yang, Yao-Hsu
Fever of unknown origin in a very old patient: beware of the kidney!
ANCA-associated vasculitis affects more than 20 per million of the population per year and prevails in the elderly. Renal involvement, either isolated or in the context of systemic vasculitis, is common. We report the case of an 86-year-old patient who presented with a histologically proven renal limited vasculitis and with fever and fatigue but with normal renal function and urine analysis. Serum creatinine increased and microscopic hematuria became evident only 3�weeks after symptoms onset, whereas ANCA positivity was the only early key to the diagnosis. This case shows that in the elderly an ANCA-related pauci-immune glomerulonephritis in its earliest stages should be suspected in the presence of a fever of unknown origin even if serum and urine analyses are normal. PMID:24569957
Giua, Renato; Pedone, Claudio; Onetti Muda, Andrea; Antonelli Incalzi, Raffaele
What's wrong with this artery? A medical disease discovered by a surgeon.
A 50-year-old man presenting with chest pain had positive stress echocardiography; and angiogram showed single artery coronary stenosis, presumed to be atherosclerotic. He was started on optimal medical therapy with good compliance. Four months later, he had a myocardial infarction (MI) and cardiac catheterisation surprisingly showed interval development of severe three-vessel stenosis. He underwent coronary artery bypass grafting (CABG), during which the cardiothoracic surgeon noticed severely inflamed coronary arteries, concerning for vasculitis. Following CABG, the patient continued to have chest pain and was admitted again for MI within 4?months of surgery. Subsequent autoimmune workup was consistent with sarcoidosis. He was started on immunosuppressive therapy for presumed sarcoid-related coronary vasculitis, and 23?months later, the patient has not developed further ischaemic events. This is a rare case and extends the clinical spectrum of cardiac sarcoidosis, presenting with rapidly progressive coronary stenosis most likely due to vasculitis, mimicking atherosclerotic coronary artery disease. PMID:25246466
Singh, Vasvi; Luthra, Saurav; Kouides, Ruth; Gadir, Abdel K
Serum cryoglobulins are found in a wide spectrum of disorders but are often transient and without clinical implications. Monoclonal cryoglobulins are usually associated with haematological disorders, whereas mixed cryoglobulins are found in many infectious and systemic disorders. So called essential mixed cryoglobulinaemia shows a striking association with hepatitis C virus (HCV) infection (> 90%). It is a systemic vasculitis (leucocytoclastic vasculitis) with cutaneous and multiple visceral organ involvement. Chronic HCV infection can lead to a constellation of autoimmune and neoplastic disorders. In this review, the aetiology, diagnosis, disease heterogeneity, and treatment of cryoglobulinaemia are discussed. PMID:11825916
Ferri, C; Zignego, A L; Pileri, S A
Takayasu Arteritis in a Young Woman
Takayasu arteritis is a chronic, progressive, autoimmune, idiopathic, large-vessel vasculitis that usually affects young adults. The disease has been reported to occur in all races and ethnicities. The diffuse nature of this vasculitis can affect multiple-organ systems to varying degrees. Herein, we report the case of a young woman whose exertional angina and claudication were the initial presentation of active Takayasu arteritis. During more than 4 years of ongoing treatment, therapy, and follow-up, she has displayed differing disease symptoms of varying intensity. We discuss the challenges of managing Takayasu arteritis in our patient and describe different treatments for this rare vasculitic disorder. PMID:19876432
Saab, Fadi; Giugliano, Robert P.; Giugliano, Gregory R.
Allertic complications of meningococcal disease. I. Clinical aspects.
Out of 717 patients with meningococcal disease 53 showed one or more of the three allergic complications: 47 (6.6%) developed arthritis, 12 (1.7%) developed cutaneous vasculitis, and 6 developed episcleritis. These complications, which were often multiple, occurred six to nine days after the beginning of the illness and three to six days after the start of successful antibiotic therapy. Those patients with severe systemic disease were prone to the complications.Histological and bacteriological study of the arthritis and vasculitis showed that these lesions were probably not due to persisting infection and suggested that they might be due to immune complex disease. PMID:4268766
Whittle, H C; Abdullahi, M T; Fakunle, F A; Greenwood, B M; Bryceson, A D; Parry, E H; Turk, J L
Local angioedema following sun exposures: a report of five cases.
Angioedema is a hereditary or acquired disease characterized by localized non-pitting swelling of the subcutaneous tissue which can affect either skin or mucous membranes. Acquired angioedema can often be related to a heterogeneous group of etiological factors including physical stimuli, although up to 38% of cases remain idiopathic. We describe 5 patients who developed an angioedema following sun exposures. All patients reported an intensely stinging angioedema strictly limited to face and extremities, when exposed to solar light. Urticarial wheals were never observed or reported by patients, and oral antihistamines proved to be of no help in preventing or improving the condition of lesions. Laboratory and phototesting data allowed ruling out all other acquired or inherited diseases characterized by photosensitivity. We propose that solar angioedema should be considered a novel clinical entity. PMID:20484931
Calzavara-Pinton, Piergiacomo; Sala, Raffaella; Venturini, Marina; Rossi, Maria Teresa; Tosoni, Cinzia; Rizzini, Fabio Lodi; Zane, Cristina
Adverse events occurring after smallpox vaccination.
We reviewed the literature on adverse events reported to occur after smallpox vaccination. Nearly one-half of the United States population is vaccinia-na�ve and may be at risk for development of serious adverse events. We describe the clinical features of postvaccinial central nervous system disease, progressive vaccinia, eczema vaccinatum, accidental implantations, "generalized vaccinia," and the common erythematous and/or urticarial rashes. In the 1960s, death occurred approximately once in every million primary vaccinations, with fatalities resulting from progressive vaccinia, postvaccinial encephalitis, and eczema vaccinatum. Death in revaccinees occurred less commonly and almost entirely from progressive vaccinia. In today's population, death rates might be higher because of the increased prevalence of immune deficiency and atopic dermatitis. PMID:12913830
Lane, J Michael; Goldstein, Joel
The first account of a bite by the New Zealand native spider Trite planiceps (Araneae: Salticidae).
New Zealand has very few arthropods that pose a threat to human health. While most New Zealand spiders are considered harmless, the bite effects of most species are unknown. Here, we describe a case of a bite by a native spider, in which a young man was bitten after rolling over in his bed. The spider was collected and identified as Trite planiceps (Salticidae, black headed jumping spider), a native species commonly encountered around homes. The initial reaction was a relatively painful, sting-like, sensation, followed by the appearance of two red puncture marks and an urticarial wheal. Symptoms eventually disappeared after 72 hours, and he has had no further dermatological problems. Trite planiceps is considered to be a rather docile spider with regard to humans, but even a docile species may still bite defensively as a last resort. Notes on this species and on treatment of spider bites are provided. PMID:20581919
Derraik, Jos� G B; Sirvid, Phil J; Rademaker, Marius
Autoimmune progesterone dermatitis: a case report.
Background. Autoimmune progesterone dermatitis is a rare cyclic premenstrual allergic reaction to progesterone produced during the luteal phase of a woman's menstrual cycle. Patients present with a variety of conditions including erythema multiforme, eczema, urticaria, angioedema, and progesterone-induced anaphylaxis. Case. Thirty-eight-year-old woman G2P2002 presents with erythema multiforme and urticarial rash one week prior to her menses starting one year after menarche. She was treated with oral contraceptive pills and the symptoms resolved. Conclusion. This is a typical case of progesterone autoimmunity. The diagnosis is based on cyclic nature of the dermatitis. This differentiates the condition from other allergies or systemic diseases with skin manifestations. Inhibition of ovulation in such cases results in decrease in progesterone secretion and prevention of symptoms. PMID:22924142
George, Rachana; Badawy, Shawky Z A
Chronic spontaneous urticaria (CU) is associated with activation of the acute phase response (APR). Nevertheless, APR-associated proteins have not been well characterized as potential biomarkers of the disease severity. To assess the pattern of complement proteins C3 and C4 � the acute phase reactants in patients with CU. C3, C4 and CRP concentrations were measured in serum of 70 patients showing different degrees of urticarial severity as well as in 33 healthy subjects. Serum C3 and C4 concentrations were significantly increased in CU patients as compared with the healthy subjects and exceed the normal lab range by about 5% and 10%, respectively. Significant differences were found between patients with mild and increased CU severity. In addition, significant correlations were observed between C3, C4 and CRP concentrations. More severe CU is characterized by higher production of C3 and C4 complements accompanied by parallel changes in CRP concentration. PMID:23706062
Background Non-infection caused urticaria is a common ailment in adolescents. Its symptoms (e.g., unusual rash appearance, limitation of daily activities, and recurrent itching) may contribute to the development of depressive stress in adolescents; the potential link has not been well studied. This study aimed to investigate the risk of major depression after a first-attack and non-infection caused urticaria. Methods This study used the Taiwan Longitudinal Health Insurance Database. A total of 5,755 adolescents hospitalized for a first-attack and non-infection caused urticaria from 2005 to 2009 were recruited as the study group, together with 17,265 matched non-urticarial enrollees who comprised the control group. Patients who had any history of urticaria or depression prior to the evaluation period were excluded. Each patient was followed for one year to identify the occurrence of depression. Cox proportional hazards models were generated to compute the risk of major depression, adjusting for the subjects� sociodemographic characteristics. Depression-free survival curves were also analyzed. Results Thirty-four (0.6%) adolescents with non-infection caused urticaria and 59 (0.3%) non-urticarial control subjects suffered a new-onset episode of major depression during the study period. The stratified Cox proportional analysis showed that the crude hazard ratio (HR) of depression among adolescents with urticaria was 1.73 times (95% CI, 1.13-2.64) than that of the control subjects without urticaria. Moreover, the HR were higher in physical (HR: 3.39, 95% CI 2.77-11.52) and allergy chronic urticaria (HR: 2.43, 95% CI 3.18-9.78). Conclusion Individuals who have a non-infection caused urticaria during adolescence are at a higher risk of developing major depression. PMID:25012668
Hereditary C3 hypocomplementemia in the rabbit.
Hereditary hypocomplementemia of the third component of complement (C3) was found in a strain of rabbits in which hereditary C8 alpha-gamma deficiency was also found. The serum C3 concentration, haemolytic C3 activity and total complement haemolytic activity (CH50) of these animals were, respectively, 6-12%, 8-13% and 27-37% of the normal levels. The haemolytic complement activity in the C3 hypocomplementemic (C3-hypo) rabbit serum was restored in a dose-dependent manner by the addition of purified rabbit C3. The levels of factor H and properdin and components C2 and C6 were in the normal range, and the levels of factors B and D and component C8 were higher than normal. The low level of serum C3 in C3-hypo rabbits was not due to C3 conversion, partial C3 antigenicity, presence of a C3 inhibitor or hypercatabolism of normal C3. Furthermore, no change in the ratio of C3 protein levels was observed between C3-hypo and normal rabbits, even after turpentine injection. In addition, the C8 alpha-gamma deficiency condition does not affect C3 activity and C3 catabolism in vivo. Mating tests showed that the C3 hypocomplementemia is transmitted as a simple autosomal co-dominant trait. C3-hypo rabbits have a lower survival at 3 months than normal rabbits. C3-hypo rabbit serum also has a lower bactericidal activity than normal rabbit serum. The PAGE under reducing conditions showed no difference in the molecular weights of C3 alpha and C3 beta chains between C3-hypo, heterozygous and normal animals. PMID:3410489
Komatsu, M; Yamamoto, K; Nakano, Y; Nakazawa, M; Ozawa, A; Mikami, H; Tomita, M; Migita, S
A case of idiopathic tracheal stenosis
Acquired tracheal stenosis is a condition that often results from trauma, neoplasm, infection, vasculitis, inflammatory or infiltrative processes. Idiopathic tracheal stenosis is a rare entity and represents a diagnosis of exclusion. We report a case of severe localized extrathoracic tracheal stenosis in a 35 year old female who was initially suspected to have asthma as the cause of several months of chronic dyspnea.
Apostolova, M.; Zeidan, B.
Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis
Infection of clinically susceptible ruminants, including domesticated cattle and American bison, with ovine herpesvirus 2 (OvHV-2) can result in the fatal lymphoproliferative and vasculitis syndrome known as malignant catarrhal fever (MCF). A reliable experimental infection model is needed to study the pathogenesis of MCF and to develop effective vaccination strategies to control the disease. An experimental aerosol infection model using
Naomi S. Taus; J. Lindsay Oaks; Katherine Gailbreath; Donald L. Traul; Donal O�Toole; Hong Li
Extrahepatic Manifestations of Chronic HCV Infection
Several extrahepatic manifestations have been reported in the natural history of hepatitis C virus infection (HCV). Up to 40-74% of patients infected with HCV might develop at least one extrahepatic manifestation during the course of their disease. Mixed Cryoglobulinemia (MC) is the most known and studied syndrome associated with HCV infection. It is a systemic vasculitis that may involve the
Alessandra Galossi; Riccardo Guarisco; Lia Bellis; Claudio Puoti
Cardiovascular and thrombophilic risk factors for central retinal vein occlusion
Retinal vein occlusion (RVO) is a relatively common disease that is often associated with a variety of systemic disorders including arterial hypertension, diabetes mellitus, dyslipidemia, and systemic vasculitis. There are various types of RVO, categorized on the basis of the site of occlusion and on the type of consequent vascular damage. Central retinal vein occlusion (CRVO) is the most frequently
Domenico Prisco; Rossella Marcucci; Laura Bertini; Anna Maria Gori
37 months), we noticed the appearance of painful perimalleolar skin ulcers. Ulcers appeared with an erythematous border, whereas the epidermidis was replaced by a fibrinous exudate and the dermis scattered by necrotic areas. Skin biopsies of the ulcerated lesions showed in all cases an histologic picture compatible with small vessel vasculitis. Circulating immune complexes were not detectable and doppler-fluximetry was
Fabio Stagno; Patrizia Guglielmo; Ugo Consoli; Paolo Fiumara; Mario Russo; Rosario Giustolisi
A Case of Henoch-Sch?nlein Purpura with P369S Mutation in MEFV Gene
Background Henoch-Sch�nlein purpura (HSP) is the most common vasculitis of childhood. HSP can affect multiple organs presenting with a characteristic rash in most of the patients. Familial Mediterranean Fever (FMF) is an inherited inflammatory disease common in mediterranean populations. HSP is the most common vasculitis seen in children with FMF. Case Presentation A 16 year old boy was referred with history of abdominal pain lasting for 20 days. He was hospitalized and had appendectomy. Due to the persistence of his abdominal pain after surgery he was admitted to our hospital. His physical examination showed palpable purpuric rashes symmetrically distributed on lower extremities. Abdominal examination revealed periumbilical tenderness. Laboratory tests showed elevated erythrocyte sedimentation rate, Creactive protein and fibrinogen. Urinalysis revealed microscopic hematuria and severe proteinuria. The fecal occult blood testing was positive. Based on these clinic findings, the patient was diagnosed as HSP with renal, gastrointestinal tract and skin involvement. We performed DNA analysis in our patient because he had diagnosis of vasculitis with severe symptoms and found that he was carrying heterozygote P369S mutation. Conclusion Our case is noteworthy as it indicates that it may be important not to overlook presence of FMF mutations in patients with a diagnosis of severe vasculitis. PMID:23056796
Ertan, Pelin; Tekin, Gokhan; Sahin, Gulseren Evirgen; Kas?rga, Erhun; Taneli, Fatma; Kandioglu, Ali Riza; Sozeri, Betul
CD40 ligand gene and Kawasaki disease
Kawasaki disease (KD) is an acute systemic vasculitis syndrome of infants and young children. Although its etiology is largely unknown, epidemiological findings suggest that genetic factors play a role in the pathogenesis of KD. To identify genetic factors, affected sib-pair analysis has been performed. One of the identified peaks was located on the Xq26 region. A recent report of elevated
Yoshihiro Onouchi; Sakura Onoue; Mayumi Tamari; Keiko Wakui; Yoshimitsu Fukushima; Mayumi Yashiro; Yoshikazu Nakamura; Hiroshi Yanagawa; Fumio Kishi; Kazunobu Ouchi; Masaru Terai; Kunihiro Hamamoto; Fumiyo Kudo; Hiroyuki Aotsuka; Yoshitake Sato; Akiyoshi Nariai; Yoichi Kaburagi; Masaru Miura; Tsutomu Saji; Tomisaku Kawasaki; Yusuke Nakamura; Akira Hata
... glomerulonephritis). Risk factors include: A personal or family history of IgA nephropathy or Henoch Schonlein purpura , a form of vasculitis that affects many parts of the body Caucasian or Asian ethnicity IgA nephropathy can occur in people of ...
Colonic localization of indium-111 labeled leukocytes in active Behcet's disease
A patient with known Behcet's disease demonstrated intense colonic localization of In-111 labeled leukocytes. Gastrointestinal involvement had not been previously manifested, but extensive colonic inflammation was documented by endoscopy. This case illustrates the utility of In-111 labeled leukocyte imaging for detecting active bowel disease in a debilitated patient with documented Behcet's vasculitis.
Harre, R.G.; Conrad, G.R.; Seabold, J.E.
[Vasculitic peripheral neuropathy].
The typical clinical manifestation of vasculitic peripheral neuropathy is sensory-dominant multiple mononeuropathy, although it can progress to distal-dominant sensorimotor polyneuropathy. It is painful in most cases. Peripheral nerves may be the most prone to produce symptoms of the vasculitis. Nerve conduction studies show reduced amplitude of M wave or sensory nerve action potential, which depends on the degree of injury of a nerve examined. Wallerian degeneration can cause pseudo-conduction block in the acute stage and temporal dispersion in the chronic stage. However, a definite diagnosis requires histological confirmation. Combined biopsy of the sural nerve and the peroneus brevis muscle can be performed by a single incision. Skin biopsy can also be performed. To increase the diagnostic yield, biopsy specimens are prepared in different manners to observe as many cross sections as possible: frozen unfixed, formalin-fixed paraffin-embedded, and glutaraldehyde-fixed epon embedded specimens, as well as teased fiber preparation of a nerve. Vasculitic peripheral neuropathy usually results from small-vessel vasculitis. There are still controversies regarding the classification of vasculitides. Differential diagnosis of vasculitis includes infection and lymphoma. Delayed diagnosis and treatment of neuropathy result in the impairment of ADL and QOL. Recovery from axonal degeneration usually takes time and is not always possible. Treatment includes corticosteroid, cyclophosphamide, and intravenous immunoglobulin administration; however, the intensity of treatment depends on the disease activity of vasculitis. PMID:24200608
Oya, Yasushi
Peripheral ulcerative keratitis (PUK) is a rare but serious inflammatory eyecondition that can complicate rheumatoid arthritis. PUK can be a warning sign of impending vasculitis, and cytotoxic therapy may be necessary to induce remission. We have encountered three patients with PUK in the past year. Two patients had long-standing quiescent rheumatoid arthritis who developed photophobia. Diagnosis was made by slit
Haitham Jifi-Bahlool; Constantine Saadeh; James O'Conner
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with pulmonary hemorrhage is rare in childhood. Standard treatment includes corticosteroids and cyclophosphamide (CYC), which is associated with a high level of toxicity. We report a white female with ANCA positive pulmonary hemorrhage who was treated with cyclophosphamide (CYC) and rituximab (RTX) combination therapy. PMID:22032441
Atrial myxoma masquerading as Takayasu�s arteritis
Lesson We describe the case of a 48-year-old woman whose atrial myxoma was mistaken for vasculitis. The case report highlights the reasons why these two disorders may become confused, the dangers of initiating the wrong treatment and a simple means of avoiding misdiagnosis.
Fung, Kenneth; Edmondson, Stephen
Enfermedad de Adamantiades-Beh�et: Un proceso enigm�tico con manifestaciones orales
Adamantiades-Beh�et disease (ABD) is a chronic multisystemic vasculitis that is able to affect any human organ or system. Recurrent oral ulcers are a very important clinical sign. ABD is a worldwide pathology, which prevalence varies according to the population and geographic location. Although ABD has been known for ages, its aetiology remains an enigma. Genetic, immunological and microbiological factors have
Asier Eguia; Mariana Villarroel; Rafael Mart�nez-Conde; Mar�a �ngeles Echebarr�a; Jos� Manuel Aguirre
Cutaneous zygomycosis caused by Saksenaea vasiformis in a diabetic patient.
A 49-year-old woman with diabetes mellitus rapidly developed necrotizing cellulitis with fat necrosis and vasculitis after minor trauma to the right arm. Zygomycosis was diagnosed histologically. The lesion responded to aggressive debridement, amphotericin B, and normalization of blood glucose. Cultures yielded structures characteristic of Saksenaea vasiformis only after transfer to saline agar. Images PMID:7929783
Bearer, E A; Nelson, P R; Chowers, M Y; Davis, C E
Superantigens and their Association with Dermatological Inflammatory Diseases: Facts and Hypotheses
Superantigens have been suggested to play an important role in the pathogenesis of several inammatory skin diseases as well as systemic diseases such as atopic dermatitis, psoriasis, vasculitis, T-cell lymphoma and autoimmune diseases. Infections often precede the onset and relapse of these diseases, and antibiotic treatment with or without additional glucocorticosteroids and immunoglobulins is occasionally successful. Superantigens are microbial proteins
Uta Jappe
UniversityofConnecticutHealthCenter 263FarmingtonAvenue
and #12;VASCUlAR DISEASE m ay 2011 | www.uchc.edu The UConn Health Center is the site of the first procedure performed in the Hartford area to repair an abdominal aneurysm without using a surgical incision. "Since there is no incision, recovery time is very short," says Dahn. "The infection rate is also lower
Oliver, Douglas L.
Small-Cell Carcinoma as a Cause of Superior Vena Cava Syndrome in a Patient with Beh�et�s Disease
Beh�et's disease (BD) is a multisystem disorder characterized by vasculitis. The first description consists of a triad of recurrent ulcers of the oral and genital mucosa with relapsing uveitis. Three forms of vascular disease (venous occlusions, arterial aneurysms and\\/or arteri- al occlusions) are found in BD (1, 2). The coexistence of neoplasia and BD is not common, and there are
Tekin Akpolat; Levent Yildiz; Ilkser Akpolat; Mustafa Bekir Selcuk; Nilg�n Ozbek; Idris Y�cel
Lingual infarction in Wegener's Granulomatosis: A case report and review of the literature
Wegener's granulomatosis (WG) is a multi-system disease, characterised by the triad of necrotising granulomata affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions are associated with up to 50% of cases, although are rare as a presenting feature. The most common oral lesions associated with WG are ulceration and strawberry gingivitis. We review the literature regarding
Lachlan M Carter; Eitan Brizman
La troponina I nella malattia di Kawasaki Troponin I in Kawasaki disease
Background. Kawasaki's disease is a multisystemic vasculitis affecting small- and medium-caliber arteries, and in 20-25% of patients coronary arteries, too. The diagnosis of the disease is clinical. In its incomplete form, not all clinical criteria are pre- sent, but heart involvement is evident. An early, sensitive and specific biochemical marker of myocardial damage could be useful in the diagnostic and
G. Cremaschini; R. Ferrari; T. Bignotti; F. Ricci; S. Fonte; L. D. Notarangelo
We describe 3 unusual cases of granuloma annulare with multiple macular lesions in a distribution that simulated mycosis fungoides in patients with no associated underlying diseases. Repeated biopsies showed typical well-formed palisading granulomas and no evidence of an atypical lymphocytic infiltrate. There was no vasculitis, neutrophilic, eosinophilic, or interstitial infiltrate. The patients had no associated underlying diseases. Most of the
Hong Wu; Alan Barusevicius; Stuart R Lessin
Alopecia areata and humpy-back syndrome in suckling piglets
This report describes an uncommon variant of humpy-back syndrome associated with multiple rib fractures and multisystemic vasculitis in several nursing piglets and, for the first time, a skin disease in swine consistent with alopecia areata. Both conditions were observed concurrently on the farm and occasionally in the same piglets. PMID:23372194
Drolet, Richard; Denicourt, Martine; D'Allaire, Sylvie
Influences of Chloride and Hypochlorite on Neutrophil Extracellular Trap Formation
such as small vessel vasculitis, lupus and cystic fibrosis lung disease. Reactive oxygen species (ROS at tissue foci of infection. NETs have also been implicated in the pathogenesis of non-infectious diseases patients with chronic granulomatous disease or myeloperoxidase (MPO) deficiency, produce fewer NETs
Nizet, Victor
Beh�et's disease in a patient with immunodeficiency virus infection
A patient with human immunodeficiency virus (HIV) infection who developed Beh�et's disease is described. As various vasculitis syndromes have been encountered recently in association with HIV infection it is suggested that Beh�et's disease may be related to the HIV infection in this patient.
D Buskila; D D Gladman; J Gilmore; I E Salit
Behcet's Disease: New Concepts in Cardiovascular Involvements and Future Direction for Treatment
Behcet's disease (BD) is the only systemic vasculitis involving both arteries and vein in any sizes. It frequently encounters in rheumatology clinics. It has some major morbidities and even fatal outcomes in some cases. The aim of this paper is to analyze the main concepts on pathophysiology and treatment options in BD, focusing on cardiovascular aspects, thrombosis, and potential future treatment. PMID:22530146
Owlia, M. B.; Mehrpoor, G.
Takayasu arteritis in children
Takayasu arteritis (TA) is a large vessel vasculitis that usually affects young female patients during the second and third decades of life, but has been reported in children as young as 24 months of age. Aim of this report was to describe four children (two girls) with TA, as well as summarizing main published studies. The mean age at presentation
Safia Al abrawi; Marine Fouillet-Desjonqueres; Louis David; Xavier Barral; Pierre Cochat; Rolando Cimaz
Thrombotic thrombocytopenic purpura in adult Still's disease.
We describe 2 patients with adult Still's disease who developed thrombotic thrombocytopenic purpura (TTP) and were successfully treated. Although TTP has been associated with autoimmune diseases, usually with systemic lupus erythematosus or various forms of vasculitis, it has rarely been observed in patients with adult Still's disease. This uncommon coexistence of 2 clinical entities may indicate similar pathogenetic mechanisms. PMID:8882053
Boki, K A; Tsirantonaki, M J; Markakis, K; Moutsopoulos, H M
Wegener�s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis
Wegener�s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener�s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener�s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener�s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener�s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener�s granulomatosis, which mimicked inflammatory bowel disease. PMID:24124396
Shahedi, Kamyar; Hanna, Ramy Magdy; Melamed, Oleg; Wilson, James
Eficacia del adalimumab en la enfermedad de Beh�et: descripci�n de 6 casos
Beh�et's disease (BD) is a systemic vasculitis, with a more aggressive course in young males. Orogenital ulcers, uveitis and cutaneous lesions are the most frequent manifestations.We analyzed the effects of adalimumab on six patients with BD pretreated with inmunosupressive therapy, two of whom had received infliximab. We observed a good clinical response in all patients. To date, after a mean
Javier Calvo Catal�; Cristina Campos Fern�ndez; Amalia Rueda Cid; Mar�a Isabel Gonz�lez-Cruz Cervellera; Aurelio Baixauli Rubio; Mar�a Dolores Pastor Cubillo
OBJECTIVES--To test the effectiveness of and tolerance to interferon-alpha 2b (INFa2b) in association with plasma exchanges for the treatment of polyarteritis nodosa (PAN) related to hepatitis B virus (HBV). METHODS--A prospective, non blinded, multicentre trial was carried out in which patients with multisystemic HBV-related PAN were included. Each patient received the association of INFa2b and plasma exchanges. The end point of the study was control of the disease (recovery or remission) or death. RESULTS--Six patients were included in the study. Each patient had histopathological or arteriographic evidence of vasculitis and was infected with actively replicating HBV. All patients were alive at the end of the study and no longer presented clinical or laboratory evidence of systemic vasculitis. HBeAg/anti-HBeAb seroconversion was observed in four patients (66.6%) and HBsAg/anti-HBsAb in 3/6 (50%). Two patients are still being treated with INFa2b because of chronic active hepatitis. CONCLUSIONS--It is considered that this new therapeutic approach to HBV-related PAN effectively cured systemic vasculitis and was associated with control of HBV infections. Antiviral therapy may have a role to play as the first line treatment regime of virus-induced vasculitis. PMID:7912504
Guillevin, L; Lhote, F; Sauvaget, F; Deblois, P; Rossi, F; Levallois, D; Pourrat, J; Christoforov, B; Trepo, C
Erythema elevatum diutinum in association with dermatitis herpetiformis.
Erythema elevatum diutinum (EED) is a rare skin disease that initially presents as leucocytoclastic vasculitis and later resolves with fibrosis. Dermatitis herpetiformis is an autoimmune blistering disease characterized by granular deposits of immunoglobulin A (IgA) in dermal papillae. We report a rare association of these two disorders. PMID:24616856
Chandrasekaran, Shanmuga Sekar; Rai, Reena; Vedachalam, Sandhya; Dorairaj, Lathika; Palaniraman, Surendran
Polyarteritis nodosa presented as a dilatation of the intrahepatic bile duct.
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of small- and medium-sized arteries in multiorgan systems. PAN may affect the gastrointestinal tract in 14%-65% of patients, but rarely involves the biliary tract and liver. We describe a patient without underlying disease who was diagnosed with PAN during resection of the gallbladder and liver. PMID:25368854
Choi, Han Lim; Sung, Ro Hyun; Kang, Min Ho; Jeon, Hyun Jeong; Yun, Hyo Yung; Jang, Lee Chan; Choi, Jae Woon; Song, Young Jin; Ryu, Dong Hee
Polyarteritis nodosa presented as a dilatation of the intrahepatic bile duct
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of small- and medium-sized arteries in multiorgan systems. PAN may affect the gastrointestinal tract in 14%-65% of patients, but rarely involves the biliary tract and liver. We describe a patient without underlying disease who was diagnosed with PAN during resection of the gallbladder and liver. PMID:25368854
Choi, Han Lim; Sung, Ro Hyun; Kang, Min Ho; Jeon, Hyun Jeong; Yun, Hyo Yung; Jang, Lee Chan; Choi, Jae Woon; Song, Young Jin
Allergic Complications of Meningococcal Disease I�Clinical Aspects
Out of 717 patients with meningococcal disease 53 showed one or more of the three allergic complications: 47 (6�6%) developed arthritis, 12 (1�7%) developed cutaneous vasculitis, and 6 developed episcleritis. These complications, which were often multiple, occurred six to nine days after the beginning of the illness and three to six days after the start of successful antibiotic therapy. Those
H. C. Whittle; M. T. Abdullahi; F. A. Fakunle; B. M. Greenwood; A. D. M. Bryceson; E. H. O. Parry; J. L. Turk
Churg-strauss syndrome in childhood: a case report.
Churg-Strauss syndrome is a rare form of small-vessel vasculitis. In the current report, we describe the case of a 17-year-old Czech girl predominantly characterized by peripheral neuropathy, the presence of cardiac and pulmonary involvement, hypereosinophilia, asthma, and sinusitis that led to the diagnosis of Churg-Strauss syndrome. PMID:24170259
Albahri, Ziad; Minxov�, Lenka; Luke�, Anton�n; Mawiri, Abdul Al; Stef�?kov�, S�rka
Between 1998 and 2001, tissues from four captive white-tailed deer were observed to have histologic lesions of systemic lymphocytic vasculitis. These lesions suggested malignant catarrhal fever, although epizootic hemorrhagic disease and bluetongue were included in the differential diagnosis. Initial diagnostic efforts, including virus isolation and reverse transcription-PCR for epizootic hemorrhagic disease virus and blue- tongue virus, failed to identify an
Steven B. Kleiboeker; Margaret A. Miller; Susan K. Schommer; Jose A. Ramos-Vara; Magalie Boucher; Susan E. Turnquist
A case of ulcerative colitis presenting as pyoderma gangrenosum and lung nodule
Pyoderma gangrenosum is a phenomenon of cutaneous ulceration where etiology is not well understood. About half of the cases have an associated extracutanoeus manifestation or associated systemic diseases. Most commonly associated systemic disorders include inflammatory bowel disease, hematologic malignancies, autoimmune arthritis, and vasculitis. We are reporting a case where pyoderma gangrenosum has presenting features for ulcerative colitis. PMID:24596646
Li, Xin; Chandra, Subhash
State of the Art in the Treatment of Systemic Vasculitides
Anti-neutrophil cytoplasm antibodies (ANCA) are associated with small vessel vasculitides (AASV) affecting the lungs and kidneys. Structured clinical assessment using the Birmingham Vasculitis Activity Score and Vasculitis Damage Index should form the basis of a treatment plan and be used to document progress, including relapse. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus azathioprine, or methotrexate. Additional plasmapheresis is effective for very severe disease, reducing dialysis dependence from 60 to 40% in the first year, but with no effect on mortality or long-term renal function, probably due to established renal damage. In milder forms of ANCA-associated vasculitis, methotrexate, leflunomide, or mycophenolate mofetil are effective. Mortality depends on initial severity: 25% in patients with renal failure or severe lung hemorrhage; 6% for generalized non-life threatening AASV but rising to 30�40% at 5?years. Mortality from GPA is four times higher than the background population. Early deaths are due to active vasculitis and infection. Subsequent deaths are more often due to cardiovascular events, infection, and cancer. We need to improve the long-term outcome, by controlling disease activity but also preventing damage and drug toxicity. By contrast, in large vessel vasculitis where mortality is much less but morbidity potentially greater, such as giant cell arteritis (GCA) and Takayasu arteritis, therapeutic options are limited. High dose glucocorticoid results in significant toxicity in over 80%. Advances in understanding the biology of the vasculitides are improving therapies. Novel, mechanism based therapies such as rituximab in AASV, mepolizumab in eosinophilic granulomatosis with polyangiitis, and tocilizumab in GCA, but the lack of reliable biomarkers remains a challenge to progress in these chronic relapsing diseases. PMID:25352843
Luqmani, Raashid Ahmed
Neutrophilic dermatosis of the dorsal hands associated with hypopharyngeal carcinoma.
Neutrophilic dermatosis of the dorsal hands (NDDH) is a rare and recently described disorder regarded as a subset of neutrophilic dermatosis, similar to superficial pyoderma gangrenosum and pustular vasculitis. Many currently consider it to be a localized variant of Sweet Syndrome. We describe the case of a 63-year-old male patient with NDDH associated with hypopharyngeal carcinoma. A biopsy demonstrated changes consistent with those seen in Sweet Syndrome, with leukocytoclasia but no signs of true vasculitis. Treatment with oral prednisolone resulted in complete resolution of the lesions in 1 month, with no recurrences. The clinical presentation, the histological features, and the excellent response to oral corticosteroid therapy allowed us to classify this case as NDDH. We emphasize the rare association of this entity with a solid tumor. PMID:18718189
Cravo, Mariana; Cardoso, Jos� C; Tellechea, Oscar; Cordeiro, Margarida R; Reis, Jos� Pedro; Figueiredo, Am�rico
Levamisole is a veterinary anthelmintic drug with immunomodulatory properties in humans. It has become increasingly common as a contaminant in cocaine and is now detected in the majority of cocaine seized in the United States. A variety of adverse reactions have been reported in association with levamisole, the most severe being agranulocytosis, vascular occlusive disease, and thrombotic vasculopathy, with or without vasculitis. The combination of rapidly progressive cutaneous ecchymosis and purpura leading to necrosis, often affecting the ears and cheeks; neutropenia or agranulocytosis; serologic autoantibodies; and thrombotic vasculopathy, with or without associated vasculitis, in a patient who has recently used cocaine is characteristic of exposure to contaminant levamisole. We report the case of a 54-year-old man who presented with the clinical findings of levamisole-contaminated cocaine use and review the literature regarding cutaneous reactions associated with levamisole. Our case highlights this important public health issue and represents a clinical course that is unusually severe. PMID:24605347
Gaertner, Erich M; Switlyk, Stephen A
[Therapy of peripheral vessel stenosis and occlusion in patients with thromboangiitis obliterans].
Vasculitis consists of a group of diseases characterized by an inflammatory process of the vessel wall. There is a wide variation in symptoms and almost any organ or tissue can be affected. Thromboangiitis obliterans (TAO; also known as Buerger's disease) is a special form of vasculitis with recurring inflammation and thrombosis of small and medium size arteries and veins of the hands and feet. To date the etiology still remains unclear but there is a strong association with the use of tobacco products. Ulcerations and gangrene of the extremities are common complications often resulting in the need for amputation of the extremity involved. Treatment of TAO includes both surgical and non-surgical methods but there is still no agreement concerning the optimal treatment strategy. In this contribution the advantages and disadvantages of different treatment options will be addressed and representative cases will be discussed. PMID:20717643
Sandner, T A; Degenhart, C; Becker-Lienau, J; Reiser, M F; Treitl, M
Kawasaki disease is a pediatric systemic vasculitis of unknown etiology for which a genetic influence is suspected. We identified a functional SNP (itpkc_3) in the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene on chromosome 19q13.2 that is significantly associated with Kawasaki disease susceptibility and also with an increased risk of coronary artery lesions in both Japanese and US children. Transfection experiments showed that the C allele of itpkc_3 reduces splicing efficiency of the ITPKC mRNA. ITPKC acts as a negative regulator of T-cell activation through the Ca2+/NFAT signaling pathway, and the C allele may contribute to immune hyper-reactivity in Kawasaki disease. This finding provides new insights into the mechanisms of immune activation in Kawasaki disease and emphasizes the importance of activated T cells in the pathogenesis of this vasculitis. PMID:18084290
Onouchi, Yoshihiro; Gunji, Tomohiko; Burns, Jane C; Shimizu, Chisato; Newburger, Jane W; Yashiro, Mayumi; Nakamura, Yoshikazu; Yanagawa, Hiroshi; Wakui, Keiko; Fukushima, Yoshimitsu; Kishi, Fumio; Hamamoto, Kunihiro; Terai, Masaru; Sato, Yoshitake; Ouchi, Kazunobu; Saji, Tsutomu; Nariai, Akiyoshi; Kaburagi, Yoichi; Yoshikawa, Tetsushi; Suzuki, Kyoko; Tanaka, Takeo; Nagai, Toshiro; Cho, Hideo; Fujino, Akihiro; Sekine, Akihiro; Nakamichi, Reiichiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Nakamura, Yusuke; Hata, Akira
Adult Dermatomyositis with Bleeding Ulcer in the Pharynx
Dermatomyositis (DM) is one of the idiopathic inflammatory myopathies caused by complement-mediated vasculopathy or vasculitis in the muscle. Although the gastrointestinal (GI) mucosa has been reported to be involved as a result of vasculitis or vasculopathy, ulceration in the pharynx is a rare manifestation of DM. A 54-year-old woman complaining of muscle weakness in the extremities, low-grade fever, and dysphagia was diagnosed as having DM. Despite medical treatment with corticosteroids and immunosuppressive agents, her DM progressed rapidly, leading to exacerbation of the dysphagia. About 3 weeks after undergoing tracheostomy as a preventive measure against aspiration, the patient developed intractable respiratory tract hemorrhage. Repeated laryngoendoscopy revealed a bleeding ulceration in the pharynx that required hemostasis with electric cautery under general anesthesia. No bleeding recurred thereafter. Histopathologically, the pharynx exhibited nonspecific inflammatory cell infiltration in the muscle tissue. This rare manifestation may be considered in cases of DM with unexplainable airway bleeding. PMID:25328739
Kusano, Junko; Takahashi, Yuka
Managing Beh�et's disease: An update on current and emerging treatment options.
Beh�et's disease is an autoinflammatory vasculitis of unknown origin characterized by recurrent oral and genital ulcers, uveitis, arthritis and skin lesions. Additionally, involvement of the gastrointestinal tract, central nervous system and large vessels may occur. The disease is prevalent in countries along the ancient Silk Road from Eastern Asia to the Mediterranean Basin. Many treatment modalities are currently available. The choice of treatment depends on organ involvement and severity of disease. Topical treatment with corticosteroids is often sufficient for mucocutaneous involvement, however for more severe disease with vasculitis or neurological involvement a more aggressive approach is warranted. Newer drugs (biologicals) influencing cytokines and thereby T-cell function are promising with an acceptable side effect profile. Unfortunately, reimbursement of the costs of biologicals for rare disease is still a problem in various countries. In this report we discuss the current treatment modalities for Beh�et's disease. PMID:19536320
van Daele, P La; Kappen, J H; van Hagen, P M; van Laar, J Am
Differential diagnosis of multiple sclerosis: contribution of magnetic resonance techniques.
It is widely accepted that magnetic resonance imaging (MRI) findings are not totally specific for the diagnosis of multiple sclerosis. White matter lesions that mimic those of multiple sclerosis may be detected in both normal volunteers and patients harbouring different diseases. Virtually all the characteristic features of multiple sclerosis are sometimes encountered in other conditions affecting predominantly the white matter. Different conditions such as vasculitis, subcortical atherosclerotic leukoencephalopathy, Lyme disease, or acute disseminated encephalomyelitis can be virtually indistinguishable from multiple sclerosis on conventional MR images. Also the FLAIR technique adds little to the differential diagnosis. The calculation of magnetisation transfer ratio (MT ratio) may be useful to better characterise some entities, such as vasculitis, from multiple sclerosis. PMID:9647278
Triulzi, F; Scotti, G
A Case of Polyarteritis Nodosa Presenting Initially as Peripheral Vascular Disease
Polyarteritis nodosa is a rare necrotizing vasculitis that can be progressive and fatal, and its initial presenting symptom may be leg claudication due to peripheral vascular ischemia. To date, there have been fewer than ten case reports of polyarteritis nodosa presenting as peripheral vascular disease. We report a case of a 38-year-old man initially diagnosed to have premature peripheral vascular disease who presented 1 year later with symptoms consistent with giant cell arteritis and subsequently developed bowel ischemia leading to a fatal outcome. Based on the autopsy and the patient�s clinical course, the final diagnosis was polyarteritis nodosa. This case illustrates the challenges in diagnosing polyarteritis nodosa and the importance of considering vasculitis in young patients presenting with atypical presentations of diseases such as peripheral vascular disease or giant cell arteritis. PMID:18560943
Parikh, Sameer; Lu, Lee
Lateral medullary stroke in patient with granulomatous polyangiitis.
Granulomatous polyangiitis (GPA), also known as Wegener granulomatosis, is a systemic antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis that infrequently affects the central nervous system. We report a 41-year-old man with lateral medullary infarction who developed rapidly progressive renal failure. He was diagnosed with GPA based on positive serum c-ANCA and antiproteinase 3 antibodies and demonstration of pauci-immune crescentic glomerulonephritis on kidney biopsy. He was treated with Coumadin, pulse steroids, cyclophosphamide, and plasmapheresis. He had resolution of his neurologic deficits and improvement in renal function. This case report highlights the importance to consider GPA vasculitis in the differential diagnosis of stroke in patients with development of acute kidney injury. PMID:24128976
Taraschenko, Olga D; Amory, Colum F; Waldman, Jonathan; Hanspal, Era K; Bernardini, Gary L
Polyarteritis nodosa and mixed cryoglobulinaemia related to hepatitis B and C virus coinfection
OBJECTIVE�To determine the responsibility of hepatitis B virus (HBV) and hepatitis C virus (HCV) and therapeutic implications in a patient who developed systemic vasculitis.?CASE REPORT�The case of a 38�year old woman who had a past history of addiction to intravenous drugs and developed systemic vasculitis after infection by HBV and HCV is described. The clinical and laboratory findings substantiated not only the diagnosis of polyarteritis nodosa (PAN) but also that of mixed cryoglobulinaemia with a monoclonal IgM? component.?CONCLUSION�Because cryoglobulins are rarely found in HBV related PAN but often associated with HCV infection, and in light of the histological findings, cryoglobulinaemia was interpreted as being secondary to HCV infection. This example of a highly complex situation emphasises the need to gather all relevant clinical, biological, histological, and complementary data so that the best treatment for overlapping of distinct vasculitides can be selected.?? PMID:11602482
Lefebvre, P; Mouthon, L; Cohen, P; Lhote, F; Guillevin, L
Polyangiitis overlap syndrome is defined as systemic vasculitis that cannot be classified into one of the well-defined vasculitic syndromes. In this report, a female patient who presented with vasculitis-like and asthmatic symptoms was diagnosed as having polyangiitis overlap syndrome of granulomatosis with polyangiitis (GPA; formerly known as Wegener's granulomatosis) and eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome). The patient fulfilled the American College of Rheumatology diagnostic criteria for GPA and EGPA. She was successfully treated with immunosuppressants and steroids and has been in remission for 20 months. It is important to establish a proper diagnosis and introduce an appropriate treatment modality in patients with this rare and serious pathology to prevent irreversible organ damage. PMID:24577169
Uematsu, Hironori; Takata, Shohei; Sueishi, Katsuo; Inoue, Hiromasa
Allergic Complications of Meningococcal Disease I�Clinical Aspects
Out of 717 patients with meningococcal disease 53 showed one or more of the three allergic complications: 47 (6�6%) developed arthritis, 12 (1�7%) developed cutaneous vasculitis, and 6 developed episcleritis. These complications, which were often multiple, occurred six to nine days after the beginning of the illness and three to six days after the start of successful antibiotic therapy. Those patients with severe systemic disease were prone to the complications. Histological and bacteriological study of the arthritis and vasculitis showed that these lesions were probably not due to persisting infection and suggested that they might be due to immune complex disease. ImagesFIG. 3FIG. 4FIG. 5FIG. 6 PMID:4268766
Whittle, H. C.; Abdullahi, M. T.; Fakunle, F. A.; Greenwood, B. M.; Bryceson, A. D. M.; Parry, E. H. O.; Turk, J. L.
Progressive pigmentary purpura.
A 58-year-old man presented for evaluation and treatment of non-tender, non-pruritic, annular patches on the right temple and frontal aspect of the scalp that reddened with exercise. A biopsy specimen showed a purpuric dermatitis with features of lymphocytic vasculitis; a diagnosis of exercise-induced progressive pigmentary purpura was made. Whereas progressive pigmentary purpura is purported to be caused by exercise, other similar appearing entities are associated with exercise, namely exercise-induced vasculitis (EIV). EIV may be considered as an acute microcirculatory deficiency and thermoregulation decompensation that occurs after episodes of exhaustive major muscular activity or after unusual or excessive exercise. The combination of age greater than 50 years, heat, and prolonged exercise are the most potent contributing factors. This is the first report of exercise-induced progressive pigmentary purpura. PMID:22031640
Brauer, Jeremy A; Mundi, Jyoti; Chu, Julie; Patel, Rishi; Meehan, Shane; Greenspan, Alan H; Stein, Jennifer
Dengue is a mosquito-borne infection caused by a flavivirus. I describe the ocular findings observed in two patients infected with dengue virus who presented with acute onset of loss of vision preceded by febrile illness, malaise, generalized fatigue headache, and maculopapular rash. Ophthalmologic evaluation in each patient revealed a normal anterior segment. Vitreous cells were noted in one patient. Ophthalmoscopy revealed multiple foci of retinochoroiditis, vasculitis, cotton-wool spots, and retinal hemorrhages. The healing of the lesion showed discrete atrophic and pigmented retinochoroiditic scars. Fluorescein angiography displayed early hypofluorescence and late hyperfluorescence suggestive of leakage. The healed scars showed late staining. The serologic testing showed elevated IgG antibodies, and one had high IgM antibodies to dengue virus. Ocular findings of dengue fever consist of multifocal areas of retinochoroiditis and may lead to loss of vision. In Saudi Arabia, dengue fever should be considered in the differential diagnosis of multifocal chorioretinal lesions and retinal vasculitis. PMID:22871625
Tabbara, Khalid
Beh�et�s disease (BD) is a systemic vasculitis with unclear etiology and pathogenesis. Although several viral and bacterial\\u000a causes have been investigated in detail for several years, of late it is widely accepted that microorganisms may play a role\\u000a as a trigger, or as a cross-reactive antigens that interfere with self-antigens, such as heat-shock proteins. Genes such as\\u000a HLA-B51, MICA and
Salih Pay; ?smail ?im?ek; Hakan Erdem; Ayhan Din�
A 2-year-old neutered male golden retriever dog presented for lameness secondary to ulcerations of multiple digital paw pads was diagnosed with vasculitis and hypercholesterolemia. Despite treatment, ischemic necrosis progressed to include all distal extremities and the dog eventually expired due to myocardial infarction secondary to severe atherosclerosis. The rapid demise and the dermatologic lesions may have been secondary to cholesterol embolism syndrome which has never before been reported in a dog. PMID:24790237
Boynosky, Nicole A.; Stokking, Laura
Low-Dose Cyclosporin for Multiple Colonic Ulcers Associated with Mixed Connective Tissue Disease
:�� This report describes a patient with multiple colonic ulcers and mixed connective tissue disease. The histological findings\\u000a of the colonic lesions showed vasculitis with T-cell infiltration, and the peripheral T cells were frequently in the activated\\u000a phase of the cell cycle. In this patient, low-dose cyclosporin treatment (2.5 mg\\/kg\\/day) inhibited the T-cell activation in\\u000a the peripheral lymphocytes and was
N. Maeda; S. Abe; T. Yoshizawa; K. Ogawa; I. Sekigawa; N. Iida; M. Eguchi; M. Matsumoto; H. Hashimoto; S. Hirose
Two Cases of Frosted Branch Angiitis with Central Retinal Vein Occlusion
Background: Frosted branch angiitis usually occurs in children, and has a good prognosis. We report two cases of unilateral frosted branch angiitis in adults. Both had poor visual outcomes because of associated central retinal vein occlusion and neovascular glaucoma.Cases: Case 1 was a 36-year-old woman. Almost all retinal veins and some retinal arteries showed vasculitis in her right eye, and
Toshikatsu Kaburaki; Makoto Nakamura; Kazuhiro Nagasawa; Miyuki Nagahara; Satoru Joko; Yujiro Fujino
Objectives: to identify the region of bactericidal\\/permeability-increasing protein (BPI) recognized by anti-BPI AN CA.Methods: sera from 140 patients with a variety of clinical diagnoses (20 systemic vasculitis, 12 cystic fibrosis, 22 bronchiectasis\\/chronic obstructive airways disease, three diabetes mellitus, 13 chronic renal failure, 12 primary sclerosing cholangitis, eight ulcerative colitis, three Crohn's disease, seven cancer, and 40 other or unknown diagnoses)
Austin C. Dunn; Russell S. Walmsley; Russell L. Dedrick; Andrew J. Wakefield; Christopher M. Lockwood
Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.
Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis that usually occurs in children younger than 2 years of age. It is a rare disease characterized by mild fever, a violent onset of hemorrhagic skin lesions, and edema usually followed by a spontaneous and complete recovery. Although the etiology is unknown, AHEI often follows infections, drug treatment, or vaccination. In the present report, the authors describe 4 cases of AHEI and review the relevant literature. PMID:18772356
Karremann, Michael; Jordan, Alexander J; Bell, Nellie; Witsch, Michael; D�rken, Matthias
Epstein-Barr virus (EBV), a ubiquitous human herpesvirus and an aetiological agent of infectious mononucleosis, has a unique trop-ism for B lymphocytes1. Clinical and laboratory features of chronic active EBV infections are chronic or persistent infectious mononu-cleosis-like symptoms and high antibody titre against early anti-gens (EA)2-4 . Kawasaki disease (KD), aetiology unknown, is thought to be self-limited immunologically mediated vasculitis. Clinical
Hideaki Kikuta; Yuichi Taguchi; Kazuhiro Tomizawa; Kimikazu Kojima; Nobuaki Kawamura; Akihito Ishizaka; Yukio Sakiyama; Shuzo Matsumoto; Shosuke Imai; Toshifumi Kinoshita; Shigeki Koizumi; Toyoro Osato; Ichiro Kobayashi; Isamu Hamada; Kanji Hirai
A patient with advanced rheumatoid arthritis and severe clinical manifestations of rheumatoid vasculitis died of acute renal failure after 30 months of treatment with high-dosage D-penicillamine. She had had no signs of adverse drug reactions until the terminal illness. Although streptococcal pharyngitis was diagnosed late in her disease, penicillamine-induced immune complex glomerular damage is considered more likely than poststreptococcal glomerulonephritis,
Seyed A. Sadjadi; Mildred S. Seelig; Adolph R. Berger; Mayer Milstoc
Peripheral neuropathy in two patients with rheumatoid arthritis receiving infliximab treatment
Antitumor necrosis alpha agents have been successfully used for the treatment of rheumatoid and seronegative arthritis, Crohn�s\\u000a disease, psoriasis, and severe cases of vasculitis. Several side effects have been observed in patients receiving these agents\\u000a including hypersensitivity reactions, infections, drug-induced lupus, or demyelinating syndromes. The presence of peripheral\\u000a neuropathy has been reported only in isolated cases. We describe two cases
Maria G. Tektonidou; John Serelis; Fotini N. Skopouli
Acute myocardial infarction and cerebrovascular accident in a young girl after a viper bite.
A 17-year-old girl developed an acute myocardial infarction immediately after being bitten by a viper and four days later she had a cerebrovascular accident. The close clinical and laboratory follow-up of this case suggested that myocardial damage could be attributed to a direct cardiotoxic effect of the venom, while the brain injury that subsequently appeared was probably the result of a disseminated intravascular coagulopathy, possibly in conjunction with vasculitis. Images PMID:7073914
Aravanis, C; Ioannidis, P J; Ktenas, J
Opinion statement��Kawasaki disease is an acute vasculitis of childhood, complicated by coronary artery aneurysms in up to 25% of affected children.\\u000a High-dose intravenous immunoglobulin (IVIG) administered in the acute phase of Kawasaki disease leads to a fivefold reduction\\u000a in the prevalence of coronary aneurysms. When fever persists, a second IVIG infusion should be administered. Rescue therapies\\u000a for IVIG-resistant Kawasaki disease
Jane W. Newburger; David R. Fulton
Kawasaki disease (KD) is an acute febrile multi-system vasculitis of unknown etiology. The diagnosis is based on clinical features. We describe a case of intravenous immunoglobulins (IVIG)-resistant KD presenting with persistent fever and massive cervical lymphadenopathy associated with mild respiratory distress. The symptoms resolved after methylprednisolone pulse therapy. High-dose pulse steroid may be an alternative therapeutic option in KD which
Hsin-Hsu Chen; Po-Mai Liu; Chin-Nam Bong; Yu-Tsun Wu; Kuender D. Yang; Chih-Lu Wang
A Proinflammatory Role of IL18 in the Development of Spontaneous Autoimmune Disease1
Serum from patients with systemic lupus erythematosus (SLE) contained significantly higher concentrations of IL-18 than normal individuals. MRL\\/lpr mice, which develop spontaneous lupus-like autoimmune disease, also had higher serum levels of IL-18 than wild-type MRL\\/ mice. Daily injections of IL-18 or IL-18 plus IL-12 resulted in accelerated proteinuria, glomerulonephritis, vasculitis, and raised levels of proinflammatory cytokines in MRL\\/lpr mice. IL-18-treated
Ehsanollah Esfandiari; Iain B. McInnes; George Lindop; Fang-Ping Huang; Max Field; Mousa Komai-Koma; Xiao-qing Wei; Foo Y. Liew
Acral gangrene as a presentation of non-uremic calciphylaxis
We are describing a case of 55-year-old obese female with significant history of uncontrolled rheumatoid arthritis, who recently had decreased her immune-suppression medications. She presented with extensive acral gangrene involving multiple fingers and toes. Clinical picture and laboratory findings were suggestive of vasculitis; however, skin biopsy established diagnosis of calciphylaxis, in settings of normal kidney function. Patient was treated with sodium thiosulfate with gradual improvement in her skin lesions. PMID:24327971
Hammadah, Muhammad; Chaturvedi, Shruti; Jue, Jennifer; Buletko, Andrew Blake; Qintar, Mohammed; Madmani, Mohammed Eid; Sharma, Prashant
Churg-Strauss Syndrome: An Update
Churg-Strauss syndrome is an uncommon disease of unknown cause described initially by Churg and Strauss in 1951. Even though\\u000a it was initially thought to be a variant of polyarteritis nodosa, its pathological, clinical, and laboratory features show\\u000a that it is related to the small vessel vasculitides, and it is now classified as an antineutrophil cytoplasmic antibody�associated\\u000a vasculitis. The presence of
Andy Abril
An Unusual Presentation of Giant Cell Arteritis
Giant cell arteritis (GCA) is a chronic vasculitis that typically presents with headache, fever and polymyalgia although atypical presentations are known. We present a case of GCA with nonproductive cough and pyrexia of unknown origin emphasizing this atypical nature of presentation. We report a rare association of GCA with granulomatous hepatitis. We also support the use of PET scanning in diagnosing and monitoring this condition. PMID:22811725
Prabhavalkar, Siddhesh; Bogusz, Pawel; Merard, Reena; Gormley, Mark
[Arthritis, erythema nodosum and genital ulcerations. Beh�et disease].
Behcet's disease is a disease of unknown etiology resting in between vasculitis, spondyloarthropathy and autoinflammatory diseases. If his predilection for the population originating from the Silk Road is well known, as are its cutaneous, ocular and vascular manifestations, this case illustrates the non-specificity of those manifestations, the diagnostic difficulties and the importance of routinely assessing for eyes and bowel diseases in this type of patient. PMID:21484713
Arnold, C; Revaz, S; Dudler, J
Beh�et disease is a systemic vasculitis characterized by recurrent oral and genital ulcers and uveitis. We describe a rare case of a 43-year-old woman with Beh�et disease who was admitted for pyrexia of unknown origin, cough, dyspnea, and chest pain. Her computerized tomography scan revealed pulmonary embolism and right ventricular thrombus. She was treated with anticoagulation for pulmonary embolism and right ventricular thrombus. She was well during her last follow-up. PMID:24436611
Xing, Weili; Swaminathan, Girider; Appadorai, Dorai Raj; Sule, Ashish Anil
Objectives. Longitudinal outcome data are important for research and are becoming part of routine clinical practice. We assessed an initial version of an electronic Short Form 36 (SF-36), a well-established health assessment questionnaire, in comparison with standard paper forms, in two specialist rheumatology clinics. Methods. Out-patients (20 with systemic lupus erythematosus and 31 with vasculitis) were randomly selected to complete
A. S. Wilson; G. D. Kitas; D. M. Carruthers; C. Reay; J. Skan; S. Harris; G. J. Treharne; S. P. Young; P. A. Bacon
Colchicine, an alkaloid found in extracts of the plants Colchicum autumnale and Gloriosa superb, is effective in the treatment of acute gout and dermatological conditions like leuko-cytoclastic vasculitis, psoriasis,\\u000a and Sweet�s syndrome. Oral administration of colchicine is associated with gastrointestinal side effects and its accumulation\\u000a in the body leads to bone marrow suppression. In the present study, an attempt has
Hardevinder Pal Singh; Puneet Utreja; Ashok Kumar Tiwary; Subheet Jain
Introduction��Metal-on-metal (MoM) soft tissue reactions or aseptic lymphocytic vasculitis-associated lesions (ALVAL) are being recognised\\u000a using metal artefact reduction (MAR) MR with increasing frequency following the advent of second generation metal-on-metal\\u000a bearings, but there is no standardised technique for reporting of MR appearances in this disease. The aim of this study was\\u000a to measure the reliability of a grading system designed
Helen Anderson; Andoni Paul Toms; John G. Cahir; Richard W. Goodwin; James Wimhurst; John F. Nolan
Chemokines are involved in recruitment and activation of hematopoietic cells at sites of infection and inflammation. The M3 gene of gHV68, a gamma-2 herpesvirus that infects and establishes a lifelong latent infection and chronic vasculitis in mice, encodes an abundant secreted protein during productive infection. The M3 gene is located in a region of the genome that is transcribed during
Infection or Inflammation and ICU Seizures
Effective treatment of seizures associated with central nervous system (CNS) infection and inflammation depends on rapid diagnosis\\u000a and early attainment of bactericidal activity in the cerebrospinal fluid with appropriate antimicrobial agents, or appropriate\\u000a management of vasculitis-induced cerebral complications. Despite the rarity of these disorders, there is nothing specific\\u000a regarding the management in the intensive care unit of seizures in these
Wendy C. Ziai
A Rare Cause of Pericardial Effusion: Giant Cell Arteritis
Giant cell arteritis is a granulomatous vasculitis characterized by medium or large sized vessel involvement. Although extracranial branches of the carotid artery are typically involved, involvement of aorta and its major branches can also be seen. Cardiac involvement has been encountered less frequently and pericardial effusion is rarely encountered. In this paper, a case has been presented in which pericardial effusion was determined during the examination and diagnosis was giant cell arteritis. PMID:24523978
Tasliyurt, Turker; Sivgin, Hakan; Bekar, Lutfu; Sahin, Safak; Uzun Kaya, Suheyla; Koseoglu, Resit Dogan; Kutluturk, Faruk; Yilmaz, Abdulkerim
An IgG3 monoclonal antibody, 6-19, derived from unmanipulated MRL\\/MpJ-lpr\\/lpr mice, exhibiting cryoglobulin and anti-IgG2a rheumatoid factor activities, induces skin leukocytoclastic vasculitis and glomerulonephritis when injected into normal mice. To determine the role of the gamma 3 heavy chain constant region in the generation of cryoglobulins and associated tissue lesions, we have established an IgG1 class switch variant, clone SS2F8, from
Thierry Fulpius; Francois Spertini; Luc Reininger; Shozo Izui
Primary Systemic Al Amyloidosis Presenting as Temporal Arteritis
Temporal arteritis is most common vasculitis in elderly and imitated by miscellaneous disorders. Temporal artery biopsy is the gold standard test in the diagnosis of giant cell arteritis (GCA). Hereby, we describe a case of a 67-year-old man who presented initially with temporal arteritis; however, a lip biopsy then revealed AL amyloidosis. In this respect, temporal artery biopsy should be performed for definitive diagnosis of GCA particularly patients with systemic symptoms and treatment resistant. PMID:24551471
Emmungil, Hakan; Kalfa, Melike; Basar?k, Burcu; Kahraman, Hasip; Tanhan, Ferhat; Yaman, Banu; Ozturk, Atiye; Erdemir, Zehra; Kandiloglu, Gulsen; Inal, Vedat; Kabasakal, Yasemin
Takayasu arteritis is a well known yet rare form of large vessel vasculitis. This review details the history, clinical features, differential diagnoses, classification, and immunology of the disorder. Suppression of inflammation and preservation of vascular competence are the aims of treatment. As with any rare disease, randomised controlled treatment trials are either lacking or based on small patient numbers, making management decisions difficult. Current evidence based treatments are presented and discussed. PMID:12101189
Johnston, S L; Lock, R J; Gompels, M M
Cutaneous manifestations associated with rheumatoid arthritis
Rheumatoid arthritis presents various cutaneous manifestations, either specific or nonspecific skin features, which are induced\\u000a by the activation of inflammatory cells (neutrophils, lymphocytes, macrophages), vasculopathy, vasculitis, acral deformity,\\u000a drugs, and so on. These include (1) specific findings, (2) findings due to vascular impairment, (3) findings due to immune\\u000a dysfunction, (4) characteristic neutrophilic conditions, and (5) miscellaneous conditions. On the other
T. Yamamoto
Cutaneous nodules in patients with rheumatoid arthritis: a case report and review of literatures
We report a case of 57-year-old Japanese woman with an overlap syndrome of both rheumatoid arthritis (RA) and autoimmune hepatitis,\\u000a who developed multiple skin nodules. An extensive biopsies of the nodules revealed rheumatoid neutrophilic dermatitis, showing\\u000a panniculitis without vasculitis, combining with granulomatous formation histopathologically. Since cutaneous nodules in patients\\u000a with RA are very complex, differential diagnosis should be done according
Tadashi Nakamura; Syu-ichi Higashi; Kunihiko Tomoda; Michishi Tsukano; Ken-ichi Iyama
Recurrent Intracranial Hemorrhage Due to Postpartum Cerebral Angiopathy Implications for Management
Background�Postpartum cerebral angiopathy as a cause of hemorrhagic stroke in young women is not well recognized. It is unknown whether this disorder represents a true inflammatory vasculitis or transient vasoconstriction related to the hormonal events of pregnancy and the postpartum period. Case Description�A 39-year-old woman presented with postpartum intracranial hemorrhage and, 32 months later, with subarachnoid hemorrhage, following normal pregnancies.
Melanie R. Ursell; Connie L. Marras; Richard Farb; David W. Rowed; Sandra E. Black; James R. Perry
EULAR\\/PReS endorsed consensus criteria* for the classification of childhood vasculitides
Background: There has been a lack of appropriate classification criteria for vasculitis in children.Objective: To develop a widely accepted general classification for the vasculitides observed in children and specific and realistic classification criteria for common childhood vasculitides (Henoch-Scho?nlein purpura (HSP), Kawasaki disease (KD), childhood polyarteritis nodosa (PAN), Wegener�s granulomatosis (WG), and Takayasu arteritis (TA)).Methods: The project was divided into two
S. Ozen; N. Ruperto; M. J. Dillon; A. Bagga; K. Barron; J. C. Davin; T. Kawasaki; C. Lindsley; R. E. Petty; A M Prieur; A Ravelli; P Woo
Identification of masqueraders of autoimmune disease in the office.
There are several rheumatologic and autoimmune disorders that can masquerade as allergic disease. Identification of these conditions in an office setting can be a challenge for the practicing allergist-immunologist. These conditions include rheumatoid and juvenile arthritis, Sjogren's syndrome, systemic lupus erythematosus, Behcet's and antiphospholipid syndromes, systemic sclerosis, vasculitis, sarcoidosis, chronic fatigue syndrome, and fibromyalgia. The article will address these topics and include clinical uses of immunologic tests for diagnosis. PMID:14763244
Frieri, Marianne
Ulcerative colitis complicating seronegative HLA-A2B27 rheumatoid arthritis with sacroiliitis
The case is reported of a 50 year old man with longstanding seronegative rheumatoid arthritis who developed ulcerative colitis. The patient also had sacroiliitis and his tissue was typed as HLA-A2-B27 several years before the bowel disease began. A possible overlap between primary inflammatory bowel disease, complications to the treatment of rheumatoid arthritis with drugs, and gastrointestinal rheumatoid vasculitis is
T Klausen; K Amris; P Helin
[Vascular involvement in Cogan's syndrome. A case report].
Vascular manifestations of Cogan's syndrome are rarely reported. We report the case of a young woman followed for typical Cogan's disease. Serious vascular involvement was found only during work-up for arterial hypertension. This case highlights potentially asymptomatic nature of extensive vasculitis affecting large and medium-sized vessels in Cogan's disease. Careful screening is required to prevent life-threatening complications. PMID:22197586
Luong Nguyen, L-B; Warzocha, U; Brillet, P-Y; Abad, S; Larroche, C; Amar, L; Frachet, B; Dhote, R
Gastrointestinal Manifestations of Beh�et�s Disease
Beh�et�s disease is a rare vasculitis diagnosed by the presence of recurrent oral ulcers and two of the following: genital\\u000a ulcers, typical eye lesions, typical skin lesions, and positive pathergy test. It is most commonly seen in countries along\\u000a the ancient silk road from Eastern Asia to the Mediterranean Basin. Young adults between the second and fourth decades of\\u000a life
Ellen C. Ebert
Juvenile rheumatoid arthritis and bronchiolitis obliterans organized pneumonia.
Diverse pleuropulmonary manifestations, including pleural effusion, rheumatoid nodulosis, fibrosis, obliterans brochiolitis, bronchiectasias, vasculitis, drug-induced lung disease, and obliterans bronchiolitis with organized pneumonia, have been described in patients with rheumatoid arthritis (RA). Bronchiolitis obliterans organized pneumonia (BOOP) is an uncommon condition described in patients with RA but not in juvenile RA (JRA). We described a patient with JRA who developed a BOOP. PMID:16362444
Sohn, Debora Ingrid; Laborde, Hugo Armando; Bellotti, Marta; Seijo, Luis
Objective. Churg-Strauss syndrome is a rare multisystem vasculitis of unknown aetiology. Due to the rarity of the disease, few single-centre case series have been described. The aim of this study was to evaluate a small series from a single Italian centre in order to describe the clinical features of the disease, the treatment and long-term follow-up. Methods. Nineteen Churg-Strauss syndrome
A. Della Rossa; C. Baldini; A. Tavoni; A. Tognetti; D. Neglia; G. Sambuceti; R. Puccini; C. Colangelo; S. Bombardieri
Familial Mediterranean fever (FMF) is an inherited auto-inflammatory disorder. Secondary AA amyloidosis is the most devastating complication of FMF. Nonamyloid renal involvements have also been reported in association with FMF, including vasculitis, focal and diffuse glomerulonephritis, and IgA nephropathy. We describe a patient with FMF and E148Q mutation who presented with massive proteinuria, elevated serum creatinine level, and acute glomerulonephritis picture. Disease remission was achieved after treatment with corticosteroids and colchicine. PMID:25362225
Ardalan, Mohammadreza; Nasri, Hamid
Use of drug therapy to manage acute cutaneous necrosis of the skin.
Acute cutaneous necrosis is defined as a sudden onset of gangrenous skin changes in the skin, associated with significant morbidity and mortality. The following diseases are included in this discussion: coumadin necrosis, heparin necrosis, brown recluse spider bite, necrotizing fasciitis, vasculitis, pyoderma gangrenosum, calciphylaxis, clotting abnormalities and embolic phenomena. The importance of early diagnosis, early distinction and early drug therapy or drug withdrawal must match the diagnosis for maximal preservation of the skin and underlying tissue. PMID:20514791
Wallace, Jill S; Hall, John C
Meningitis due to non-typhi salmonella is infrequent in HIV-positive adults. We report a case of a patient with >300 CD4+ cells/mm3 who presented with five episodes of recurrent meningitis, focal subarachnoid hemorrhage and cerebral vasculitis ultimately attributed to Salmonella choleraesuis infection. Even within the cART era invasive salmonellosis can occur in unusual ways in HIV-infected patients. PMID:21772932
Belloso, Waldo H; Romano, Marina; Greco, Graciela S; Davey, Richard T; Perelsztein, Ariel G; S�nchez, Marisa L; Ajzenszlos, Mart�n R; Otegui, In�s M
Two cases of rheumatoid nodules evaluated by fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) and video-assisted thoracic surgery (VATS) biopsy are reported. The first case was that of a 44-year-old woman who presented with a cavitated nodule with intense standardized uptake values (SUVs) both in the early (max 3.4) and delayed (max 4.4) phases, suggesting malignancy. However, after VATS biopsy, she was diagnosed as having a rheumatoid nodule with vasculitis. The second case was that of a 74-year-old woman admitted with bilateral lung nodules, two of which showed intense early (max 2.2) and delayed (max 6.0) phase SUVs, and mild early (max 0.6) and delayed (max 0.9) phase SUVs. These two nodules were finally proven to be a lung cancer and rheumatoid nodule without vasculitis, respectively. These cases show that rheumatoid nodules with an enhanced inflammatory process, such as vasculitis, can appear false-positive for malignancy on FDG-PET/CT scan images. PMID:22669597
Saraya, Takeshi; Tanaka, Ryota; Fujiwara, Masachika; Koji, Hitoshi; Oda, Miku; Ogawa, Yukari; Nagatomo, Tomoko; Watanabe, Masato; Yokoyama, Takuma; Ishii, Haruyuki; Takei, Hidefumi; Goya, Tomoyuki; Takizawa, Hajime; Goto, Hajime
This study was performed to evaluate pathology of experimental Encephalitozoon cuniculi (Iraqi isolate) infection in normal and immunosuppressed mice. Pathological changes were not seen in negative control mice while secondary bacterial infections were noted in the lungs, kidneys, and heart of mice given dexamethasone. Typical E. cuniculi infection lesions were found in brain, livers, lungs, and kidneys of mice given 10(7)??E. cuniculi spores/mouse orally. These lesions were in the form of nonsuppurative meningoencephalitis with vasculitis in brain, interstitial inflammation with infiltration of both lymphocytes and plasma cells in lung tissue, and nonsuppurative interstitial (focal and diffuse) nephritis, presence of vacuole containing mature and immature spores in enterocytes within the tips of villi, and lymphoiod hyperplasia of the white pulp and vasculitis of the intratrabecular vessels. Mice that were given 10(7)??E. cuniculi spores/mouse orally showed lesions similar to those observed in the previous group (vasculitis and granulomas) but the lesions were more severe and widespread. In conclusion, this is the first report of experimental E. cuniculi infection induced by E. cuniculi isolated from a naturally infected rabbit in Iraq and that infection became more severe and widespread upon the administration of dexaethasone. PMID:24772366
Al-Sadi, Hafidh I; Al-Mahmood, Saevan S
Spontaneous and antiviral-induced cutaneous lesions in chronic hepatitis B virus infection
AIM: To describe spontaneous, or interferon (IFN)- or immunization-induced skin lesions in hepatitis B virus (HBV) infection. METHODS: A comprehensive literature search of all the papers presenting case reports of dermatological lesions in patients with chronic HBV infection was carried out. We included only patients with histologically proven skin lesions that appeared in the normal course of hepatitis B infection, or after immunization for hepatitis B or antiviral treatment. RESULTS: We found 44 papers on this topic, reporting 151 cases. About 2% of patients with hepatitis B infection, mainly men, presented with skin lesions. Among patients with chronic hepatitis B, vasculitis and essential mixed cryoglobulinemia seemed to be the most frequent skin lesion (53.3%), followed by papular changes, rashes and Gianotti-Crosti syndrome, skin carcinoma and Henoch-Sch�nlein purpura were rare. IFN treatment seemed to be effective against HBV-associated and immunoglobulin-complex-mediated disease (vasculitis). Two cutaneous lesions (lichen planus and granuloma annulare) were described after hepatitis B vaccination. Systemic lupus and lupus-like lesions were the most frequently encountered lesions after antiviral treatment. Immunosuppressive and steroid therapy ameliorates lichen planus lesions in 50% of cases. CONCLUSION: Vasculitis was the most frequent spontaneous skin lesion found in chronic hepatitis B. Lichen planus was most frequent after immunization and lupus/lupus-like lesions after IFN.
Grigorescu, Ioana; Dumitrascu, Dan Lucian
This study was performed to evaluate pathology of experimental Encephalitozoon cuniculi (Iraqi isolate) infection in normal and immunosuppressed mice. Pathological changes were not seen in negative control mice while secondary bacterial infections were noted in the lungs, kidneys, and heart of mice given dexamethasone. Typical E. cuniculi infection lesions were found in brain, livers, lungs, and kidneys of mice given 107??E. cuniculi spores/mouse orally. These lesions were in the form of nonsuppurative meningoencephalitis with vasculitis in brain, interstitial inflammation with infiltration of both lymphocytes and plasma cells in lung tissue, and nonsuppurative interstitial (focal and diffuse) nephritis, presence of vacuole containing mature and immature spores in enterocytes within the tips of villi, and lymphoiod hyperplasia of the white pulp and vasculitis of the intratrabecular vessels. Mice that were given 107??E. cuniculi spores/mouse orally showed lesions similar to those observed in the previous group (vasculitis and granulomas) but the lesions were more severe and widespread. In conclusion, this is the first report of experimental E. cuniculi infection induced by E. cuniculi isolated from a naturally infected rabbit in Iraq and that infection became more severe and widespread upon the administration of dexaethasone. PMID:24772366
Al-Sadi, Hafidh I.; Al-Mahmood, Saevan S.
Diagnosis and classification of autoimmune orchitis.
Autoimmune orchitis is characterized by testis inflammation and the presence of specific antisperm antibodies (ASA). It is classified in two categories. Primary autoimmune orchitis is defined by infertility and asymptomatic orchitis associated with ASA (100%) directed to the basement membrane or seminiferous tubules in infertile men, without any systemic disease and usually asymptomatic. Secondary autoimmune orchitis is characterized by symptomatic orchitis and/or testicular vasculiti`s associated with a systemic autoimmune disease, particularly vasculitis. These patients typically demonstrate testicular pain, erythema and/or swelling. ASA in secondary autoimmune orchitis have been reported in up to 50% of patients, especially in systemic lupus erythematosus patients. The pathogenesis of primary as well as secondary autoimmune orchitis is still unknown. Although the etiology is likely to be multifactorial, testicular inflammation, infection or trauma may induce T cell response with pro-inflammatory cytokine production with a consequent blood-testis-barrier permeability alteration, ASA production and apoptosis of spermatocytes and spermatids. ASA is known to cause immobilization and/or agglutination of spermatozoa, which may block sperm-egg interaction resulting in infertility. Assisted reproduction has been used as an efficient option in primary cases and immunosuppressive therapy for secondary autoimmune orchitis, although there is no double-blind, randomized trial to confirm the efficacy of any treatment regimens for these conditions. PMID:24424181
Silva, C A; Cocuzza, M; Carvalho, J F; Bonf�, E
Basophil activation test with food additives in chronic urticaria patients.
The role of food additives in chronic urticaria (CU) is still under investigation. In this study, we aimed to explore the association between food additives and CU by using the basophil activation test (BAT). The BAT using 15 common food additives was performed for 15 patients with CU who had a history of recurrent urticarial aggravation following intake of various foods without a definite food-specific IgE. Of the 15 patients studied, two (13.3%) showed positive BAT results for one of the tested food additives. One patient responded to monosodium glutamate, showing 18.7% of CD203c-positive basophils. Another patient showed a positive BAT result to sodium benzoate. Both patients had clinical correlations with the agents, which were partly determined by elimination diets. The present study suggested that at least a small proportion of patients with CU had symptoms associated with food additives. The results may suggest the potential utility of the BAT to identity the role of food additives in CU. PMID:24527415
Kang, Min-Gyu; Song, Woo-Jung; Park, Han-Ki; Lim, Kyung-Hwan; Kim, Su-Jung; Lee, Suh-Young; Kim, Sae-Hoon; Cho, Sang-Heon; Min, Kyung-Up; Chang, Yoon-Seok
[Urticaria: diagnosis and treatment].
Urticaria is a common inflammatory skin disease. It is clinically defined as the occurrence of transient papular skin and/or mucosal lesions or subcutaneous lesions called angioedema. Chronic urticaria is defined as a clinical course over more than 6weeks. Different clinical forms of urticaria can coexist in the same patient. Urticaria results of mast cell activation. The diagnosis of urticaria is based on clinical examination. An allergic etiology for acute urticaria, although rare, is always to find and remove. Chronic urticaria is not allergic. Diagnosis is based on questioning and a careful clinical examination to rule out differential diagnoses. Few diagnostic tests are necessary for diagnosis and management, and are especially useful in case of doubtful diagnosis. The treatment of urticaria is symptomatic and based on anti-H1 second generation antihistamines as first-line therapy. In some chronic urticarial, antihistamines up dosing may be necessary. In the majority of patients, this treatment is sufficient to control chronic urticaria. In case of antihistamines failure, other treatment particularly immunomodulatory treatments can be offered in specialized departments. PMID:24581819
Soria, A; Franc�s, C
Basophil Activation Test with Food Additives in Chronic Urticaria Patients
The role of food additives in chronic urticaria (CU) is still under investigation. In this study, we aimed to explore the association between food additives and CU by using the basophil activation test (BAT). The BAT using 15 common food additives was performed for 15 patients with CU who had a history of recurrent urticarial aggravation following intake of various foods without a definite food-specific IgE. Of the 15 patients studied, two (13.3%) showed positive BAT results for one of the tested food additives. One patient responded to monosodium glutamate, showing 18.7% of CD203c-positive basophils. Another patient showed a positive BAT result to sodium benzoate. Both patients had clinical correlations with the agents, which were partly determined by elimination diets. The present study suggested that at least a small proportion of patients with CU had symptoms associated with food additives. The results may suggest the potential utility of the BAT to identity the role of food additives in CU. PMID:24527415
Kang, Min-Gyu; Song, Woo-Jung; Park, Han-Ki; Lim, Kyung-Hwan; Kim, Su-Jung; Lee, Suh-Young; Kim, Sae-Hoon; Cho, Sang-Heon; Min, Kyung-Up
Dermatoses of Pregnancy - Clues to Diagnosis, Fetal Risk and Therapy
The specific dermatoses of pregnancy represent a heterogeneous group of pruritic skin diseases that have been recently reclassified and include pemphigoid (herpes) gestationis, polymorphic eruption of pregnancy (syn. pruritic urticarial papules and plaques of pregnancy), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. They are associated with severe pruritus that should never be neglected in pregnancy but always lead to an exact work-up of the patient. Clinical characteristics, in particular timing of onset, morphology and localization of skin lesions are crucial for diagnosis which, in case of pemphigoid gestationis and intrahepatic cholestasis of pregnancy, will be confirmed by specific immunofluorescence and laboratory findings. While polymorphic and atopic eruptions of pregnancy are distressing only to the mother because of pruritus, pemphigoid gestationis may be associated with prematurity and small-for-date babies and intrahepatic cholestasis of pregnancy poses an increased risk for fetal distress, prematurity, and stillbirth. Corticosteroids and antihistamines control pemphigoid gestationis, polymorphic and atopic eruptions of pregnancy; intrahepatic cholestasis of pregnancy, in contrast, should be treated with ursodeoxycholic acid. This review will focus on the new classification of pregnancy dermatoses, discuss them in detail, and present a practical algorithm to facilitate the management of the pregnant patient with skin lesions. PMID:21909194
Anaphylactic reactions to tolperisone (Mydocalm).
Four patients with anaphylaxis attributed to the intake of the centrally acting muscle relaxant tolperisone hydrochloride (Mydocalm) were observed at the Emergency Department of the Geneva University Hospital between November 2001 and March 2003. All patients were middle-aged women who took tolperisone for chronic muscular pain. All reactions occurred within an hour after oral intake of this drug frequently prescribed in Switzerland. The severity of anaphylaxis ranged from urticarial reactions to shock with arterial hypotension. Prick-to-prick skin testing performed in one patient with a tablet of tolperisone diluted in water was negative. Its globally restricted commercialisation may explain the lack of reports on such adverse effects in the MedLine database. Anaphylactic reactions to this drug, however, are mentioned in other sources such as the Swiss Drug Compendium and the WHO drug reaction database. Together, these findings suggest that anaphylaxis to tolperisone is not uncommon and should be known to physicians in countries where this drug is available. PMID:12947534
Ribi, Camillo; Vermeulen, Christophe; Hauser, Conrad
Background While a number of the consequences of mast cell degranulation within tissues have been documented including tissue-specific changes such as bronchospasm and the subsequent cellular infiltrate, there is little known about the immediate effects of mast cell degranulation on the associated vasculature, critical to understanding the evolution of mast cell dependent inflammation. Objective To characterize the microcirculatory events that follow mast cell degranulation. Methodology/Principal Findings Perturbations in dermal blood flow, temperature and skin color were analyzed using laser-speckle contrast imaging, infrared and polarized-light colorimetry following cold-hand immersion (CHI) challenge in patients with cold-induced urticaria compared to the response in healthy controls. Evidence for mast cell degranulation was established by documentation of serum histamine levels and the localized release of tryptase in post-challenge urticarial biopsies. Laser-speckle contrast imaging quantified the attenuated response to cold challenge in patients on cetirizine. We found that the histamine-associated vascular response accompanying mast cell degranulation is rapid and extensive. At the tissue level, it is characterized by a uniform pattern of increased blood flow, thermal warming, vasodilation, and recruitment of collateral circulation. These vascular responses are modified by the administration of an antihistamine. Conclusions/Significance Monitoring the hemodynamic responses within tissues that are associated with mast cell degranulation provides additional insight into the evolution of the acute inflammatory response and offers a unique approach to assess the effectiveness of treatment intervention. PMID:23451084
Meyer, Joseph; Gorbach, Alexander M.; Liu, Wei-Min; Medic, Nevenka; Young, Michael; Nelson, Celeste; Arceo, Sarah; Desai, Avanti; Metcalfe, Dean D.; Komarow, Hirsh D.
Jellyfish sting may result in a wide range of symptoms from common erythematous urticarial eruptions to the rare box-jelly induced acute respiratory failure. In Taiwan, with the increasing frequency of international travel, cases of jellyfish sting to foreigners are on the rise. We report a case of jellyfish sting with the rare presentation of painless contact dermatitis. A 38-y-o man accidentally stepped on a sea urchin with his right foot during scuba diving in a beach in Thailand. Traditional therapy with vinegar was applied on the lesion. However, when he returned to Taiwan, erythematous patches on the left thigh with linear radiations to the leg were discovered. The skin lesions had bizzare shapes and showed progressive change. No pain or numbness was noticed. Jellyfish stingwas suspected, topical medications were applied, and the patient recovered without complication. Jellyfish stings usually result in a painful erythematous eruption. In this case, though the lesion involved a large surface, there was no pain. Delayed diagnosis of jellyfish sting was due to the atypical presentation and the physician's unfamiliarity to the Thai jellyfish sting. Awareness to the wide spectrum of jellyfish sting symptoms should be promoted. PMID:11474731
Lee, N S; Wu, M L; Tsai, W J; Deng, J F
[Allergic contact urticaria caused by a chameleon. Expression of sensitization to Ficus benjamina].
A 31-year-old man presented with a long history of rhinoconjunctivitis and sneezing that lasted from March to May. The man kept a chameleon (Chamaeleo calyptratus) as a pet and reported about recurrent urticarial lesions, erythema, and itch after skin contact with the animal. The reactions started within a few minutes and were restricted specifically to the contact sites with the animal's claws. No comparable reactions occurred in other subjects. Allergy testing confirmed allergy to hazel, alder, birch, and ash pollen, and additionally revealed sensitization to house dust mite, cat, and Ficus benjamina. Apparently the contact dermatitis originated from passive transfer of Ficus benjamina allergens to the patient's skin by the reptile which habitually climbed on a big Ficus benjamina tree in the patient's home, thereby contaminating its claws with the plant's allergenic milky sap. Careful examination revealed strong perforation of many leaves by the pointed claws. The patient denied respiratory symptoms from Ficus benjamina and intolerance of Ficus-associated fruits. PMID:15657732
Sesztak-Greinecker, G; Hemmer, W; G�tz, M; Jarisch, R
[Ficus benjamina, a perennial inhalation allergen of increasing importance].
12 patients are described with perennial allergic rhinoconjunctivitis or asthma, 3 had additionally urticarial eruptions and one edema of the eyelids. In all of them sensitization to Ficus benjamina was identified by skin prick tests, in 7 cases by the additional presence of specific IgE in vitro, and in 2 it was verified by a conjunctival provocation test. 10 patients were atopic with sensitization to other respiratory allergens such as pollen, house dust mite or animal dander. 2 patients, however, with a massive exposure to Ficus benjamina at home, were non-atopic with monovalent sensitization to this plant. Ficus benjamina is a tropical tree of the genus Ficus which belongs to the Moraceae family. The allergen is located in the plant sap, the so called latex, which after evaporation binds to dust particles on the leaf surface. Upon contact they are emanated from the leaves, thus imitating allergy to house dust components. Since Ficus benjamina is a very popular decorative plant, an increasing number of sensitized individuals is to be expected. The identification of a preventable respiratory allergen is of considerable importance for the patients. PMID:8322050
Bircher, A J; W�thrich, B; Langauer, S; Schmid, P
Review: dermatitis herpetiformis.
Dermatitis herpetiformis (DH) or Duhring-Brocq disease is a chronic bullous disease characterized by intense itching and burning sensation in the erythematous papules and urticarial plaques, grouped vesicles with centrifuge growth, and tense blisters. There is an association with the genotypes HLA DR3, HLA DQw2, found in 80-90% of cases. It is an IgA-mediated cutaneous disease, with immunoglobulin A deposits appearing in a granular pattern at the top of the dermal papilla in the sublamina densa area of the basement membrane, which is present both in affected skin and healthy skin. The same protein IgA1 with J chain is found in the small intestinal mucosa in patients with adult celiac disease, suggesting a strong association with DH. Specific antibodies such as antiendomysium, antireticulina, antigliadin and, recently identified, the epidermal and tissue transglutaminase subtypes, as well as increased zonulin production, are common to both conditions, along with gluten-sensitive enteropathy and DH. Autoimmune diseases present higher levels of prevalence, such as thyroid (5-11%), pernicious anemia (1-3%), type 1 diabetes (1-2%) and collagen tissue disease. The chosen treatment is dapsone and a gluten-free diet. PMID:24068131
Mendes, Fernanda Berti Rocha; Hissa-Elian, Adaucto; Abreu, Marilda Aparecida Milanez Morgado de; Gon�alves, Virg�nica Scaff
Sj�gren's syndrome presenting with hypereosinophilia, lymphopenia and circulating immune complexes.
Although mild peripheral eosinophilia is a common finding in Sj�gren's syndrome (SS), severe eosinophilia with a clinical picture simulating hypereosinophilic syndrome is extremely rare. We report a 24 year old male with SS presenting with swelling of the parotid glands, redness and irritation of the eyes, polyarthralgias and polyarthritis, weight loss, exertional dyspnea, malaise, erythematous and urticarial skin lesions and enlarged lymph nodes. Laboratory tests showed hypereosinophilia (34%, total 3800/mm3), lymphopenia (2%, total 220/mm3), a positive RA factor (1:2560) and decreased C3 and C4. Biopsy of an enlarged submaxillary gland was consistent with SS. A Schirmer test showed decreased tear production. Salivary glands showed a marked decrease in uptake of radioactive (Tc99) dye. Circulating immune complexes (CIC) were markedly elevated by both C1q binding and Raji cell assays. T-cell subsets showed OKT3 = 63%, OKT4 = 32% and OKT8 = 16%. "Histamine trap" in vivo test for CIC revealed fluorescence in upper dermal blood vessels with IgM, C1q, C3 and fibrin. Biopsies of the liver, bone marrow and skin revealed eosinophilic infiltration. A notable response to therapy with high doses of corticosteroids was seen with recurrence of symptoms and laboratory abnormalities after the therapy was stopped. In conclusion, we present a case of SS which is remarkable for the age and sex of the patient, extreme hypereosinophilia, marked lymphopenia, and CIC. PMID:6241858
Farnam, J; Jorizzo, J L; Grant, J A; Lavastida, M T; Ichikawa, Y; Daniels, J C
Wegener granulomatosis (granulomatosis with polyangiitis): evolving concepts in treatment.
Wegener granulomatosis (WG), the most common of the pulmonary granulomatous vasculitides, typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. THE TERM GRANULOMATOSIS WITH POLYANGIITIS (WEGENER) WAS RECENTLY PROPOSED TO REPLACE THE OLDER TERM, WG. THE TERM GRANULOMATOSIS WITH POLYANGIITIS CAN BE ABBREVIATED TO GPA, WITH THE IDEA THAT THE EPONYM WEGENER WOULD BE OMITTED OVER TIME. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive "geographic" necrosis, and granulomatous inflammation. Clinical manifestations of WG are protean; virtually any organ can be involved. The spectrum and severity of the disease are heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis. The pathogenesis of WG has not been elucidated, but both cellular and humoral components are involved. Circulating antibodies against cytoplasmic components of neutrophils [anti-neutrophil cytoplasmic antibodies (c-ANCAs)] likely play a role in the pathogenesis, and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids (CSs) is the mainstay of therapy for generalized, multisystemic WG. Historically, the combination of CYC plus CS was used for a minimum of 12 months, but concern about late toxicities associated with CYC has led to novel treatment approaches. Currently, short-course (3 to 6 months) induction treatment with CYC plus CS, followed by maintenance therapy with less toxic agents (e.g., methotrexate, azathioprine) is recommended. Further, methotrexate combined with CS may be adequate for limited, non-life-threatening WG. Recent studies suggest that rituximab may be useful for induction therapy or CYC-refractory WG. The role of other immunomodulatory agents (including trimethoprim-sulfamethoxazole) is also explored. PMID:21674414
Lynch, Joseph P; Tazelaar, Henry
Acute Retinal Necrosis in Childhood
Background Acute retinal necrosis (ARN) is a viral syndrome consisting of uveitis/vitritis, occlusive vasculitis and peripheral necrosis. Few incidents are reported in children. The etiology is reactivated herpes simplex virus (HSV) or varicella-zoster virus (VZV). Treatment with acyclovir is often used. The administration of oral glucocorticosteroids is of unproven benefit. Prognosis is variable but poor. Methods Three weeks after contracting mild chickenpox, a healthy 4-year-old girl developed blurred vision in her right eye. Severely reduced visual acuity was noted, together with anterior uveitis, �mutton-fat� precipitates and vitral flare. Retinal vasculitis with necrosis was present. Serology for toxoplasma, cytomegalovirus and HIV was negative, while HSV and VZV IgG antibodies were positive. She was treated with 30 mg/kg of intravenous methylprednisolone (3 days), 30 mg of oral prednisone (3 days), and tapering for 8 weeks. Intravenous acyclovir was given for 10 days, followed by oral acyclovir for 4 months. Aspirin (100 mg/day) was given for 4 months. Results At 12 months, the girl felt good. Her right eye acuity was 6/9, with an intraocular pressure of 17 mm Hg. The peripheral retina showed scarring but no detachment. Conclusions This is the first report of a once-daily high-dose methylprednisolone pulse therapy in one of the youngest known ARN cases. Pulsed steroid therapy was based on its known effectiveness in vasculitis, which is the main pathophysiology in ARN. There was no evidence of steroid-related viral over-replication. Our case achieved an excellent clinical and ophthalmic recovery in spite of the poor prognosis. The positive result of this case report provides a basis for further evaluation of high-dose steroid pulse therapy in ARN. PMID:24932179
Pikkel, Yoav Y.; Pikkel, Joseph
Central nervous system involvement in pediatric rheumatic diseases: current concepts in treatment.
Central nervous system (CNS) manifestations are not rare in pediatric rheumatic diseases. They may be a relatively common feature of the disease, as in systemic lupus erythematosus (SLE) and Beh�et's disease. Direct CNS involvement of a systemic rheumatic disease, primary CNS vasculitis, indirect involvement secondary to hypertension, hypoxia and metabolic changes, and drug associated adverse events may all result in CNS involvement. We have reviewed the CNS manifestations of SLE, Beh�et's disease, Henoch-Sch�nlein purpura, polyarteritis nodosa, juvenile idiopathic arthritis, juvenile ankylosing spondylitis, familial Mediterranean fever, scleroderma, sarcoidosis, Wegener's granulomatosis, Takayasu's arteritis, CINCA syndrome, Kawasaki disease, and primary CNS vasculitis; and adverse CNS effects of anti-rheumatic drugs in pediatric patients. The manifestations are diverse; ranging from headache, seizures, chorea, changes in personality, depression, memory and concentration problems, cognitive impairment, cerebrovascular accidents to coma, and death. The value of cerebrospinal fluid (CSF) examination (pleocytosis, high level of protein), auto-antibodies in serum and CSF, electroencephalography, neuroimaging with computerized tomography, magnetic resonance imaging, SPECT, PET, and angiography depends on the disease. Brain biopsy is gold standard for the diagnosis of CNS vasculitis, however it may be inconclusive in 25% of cases. A thorough knowledge of the rheumatic diseases and therapy-related adverse events is mandatory for the management of a patient with rheumatic disease and CNS involvement. Severe CNS involvement is associated with poor prognosis, and high mortality rate. High dose steroid and cyclophosphamide (oral or intravenous) are first choice drugs in the treatment; plasmapheresis, IVIG, thalidomide, and intratechal treatment may be valuable in treatment-resistant, and serious cases. PMID:18537653
Duzova, Ali; Bakkaloglu, Aysin
Early-Onset Stroke and Vasculopathy Associated with Mutations in ADA2
BACKGROUND We observed a syndrome of intermittent fevers, early-onset lacunar strokes and other neurovascular manifestations, livedoid rash, hepatosplenomegaly, and systemic vasculopathy in three unrelated patients. We suspected a genetic cause because the disorder presented in early childhood. METHODS We performed whole-exome sequencing in the initial three patients and their unaffected parents and candidate-gene sequencing in three patients with a similar phenotype, as well as two young siblings with polyarteritis nodosa and one patient with small-vessel vasculitis. Enzyme assays, immunoblotting, immunohistochemical testing, flow cytometry, and cytokine profiling were performed on samples from the patients. To study protein function, we used morpholino-mediated knockdowns in zebrafish and short hairpin RNA knockdowns in U937 cells cultured with human dermal endothelial cells. RESULTS All nine patients carried recessively inherited mutations in CECR1 (cat eye syndrome chromosome region, candidate 1), encoding adenosine deaminase 2 (ADA2), that were predicted to be deleterious; these mutations were rare or absent in healthy controls. Six patients were compound heterozygous for eight CECR1 mutations, whereas the three patients with polyarteritis nodosa or small-vessel vasculitis were homozygous for the p.Gly47Arg mutation. Patients had a marked reduction in the levels of ADA2 and ADA2-specific enzyme activity in the blood. Skin, liver, and brain biopsies revealed vasculopathic changes characterized by compromised endothelial integrity, endothelial cellular activation, and inflammation. Knockdown of a zebrafish ADA2 homologue caused intracranial hemorrhages and neutropenia � phenotypes that were prevented by coinjection with nonmutated (but not with mutated) human CECR1. Monocytes from patients induced damage in cocultured endothelial-cell layers. CONCLUSIONS Loss-of-function mutations in CECR1 were associated with a spectrum of vascular and inflammatory phenotypes, ranging from early-onset recurrent stroke to systemic vasculopathy or vasculitis. (Funded by the National Institutes of Health Intramural Research Programs and others.) PMID:24552284
Zhou, Q.; Yang, D.; Ombrello, A.K.; Zavialov, Andrey V.; Toro, C.; Zavialov, Anton V.; Stone, D.L.; Chae, J.J.; Rosenzweig, S.D.; Bishop, K.; Barron, K.S.; Kuehn, H.S.; Hoffmann, P.; Negro, A.; Tsai, W.L.; Cowen, E.W.; Pei, W.; Milner, J.D.; Silvin, C.; Heller, T.; Chin, D.T.; Patronas, N.J.; Barber, J.S.; Lee, C.-C.R.; Wood, G.M.; Ling, A.; Kelly, S.J.; Kleiner, D.E.; Mullikin, J.C.; Ganson, N.J.; Kong, H.H.; Hambleton, S.; Candotti, F.; Quezado, M.M.; Calvo, K.R.; Alao, H.; Barham, B.K.; Jones, A.; Meschia, J.F.; Worrall, B.B.; Kasner, S.E.; Rich, S.S.; Goldbach-Mansky, R.; Abinun, M.; Chalom, E.; Gotte, A.C.; Punaro, M.; Pascual, V.; Verbsky, J.W.; Torgerson, T.R.; Singer, N.G.; Gershon, T.R.; Ozen, S.; Karadag, O.; Fleisher, T.A.; Remmers, E.F.; Burgess, S.M.; Moir, S.L.; Gadina, M.; Sood, R.; Hershfield, M.S.; Boehm, M.; Kastner, D.L.; Aksentijevich, I.
Unfamiliar Manifestations of Anti-tubercular Therapy
Cutaneous drug reactions are frequent in hospitalized patients and vary from simple manifestations like rash and erythema to severe life threatening conditions like angio-oedema, erythroderma, Stevens-Johnson syndrome and toxic epidermal necrolysis. However drug eruptions with antitubercular drugs are largely unknown except few case reports. We highlight here one similar case which presented with pleomorphic cutaneous manifestations after taking anti tubercular therapy and closely mimicked vasculitis. But when the offending drugs were stopped the lesions disappeared and the patient improved. PMID:24791242
Aggarwal, Ramesh; Dwivedi, Shridhar; Aggarwal, Meenakshi
Cardiac involvement in Churg�Strauss syndrome is common and represents the main cause of mortality. We report the case of a patient with Churg�Strauss vasculitis, mitral regurgitation with left ventricular dysfunction, paroxysmal atrial fibrillation and refractory angina with non-significant coronary lesions. Cardiac denervation was proposed as an associated procedure to treat angina. The total removal of peri-adventitial and adventitial tissue around the superior vena cava, ascending aorta and main pulmonary trunk was performed. After 3 months of follow-up, the patient was angina-free and could resume his normal lifestyle. PMID:22493151
Cura, Leandro; Dayan, Victor; Cristar, Florencia; Soca, Gerardo
A 32-year-old man was admitted after bone marrow transplantation because of hematochezia. He had history of chronic graft-versus-host disease (GVHD) of the skin and the liver, and cytomegaloviral pneumonia. Barium enema and colonoscopy showed multiple colon ulcers in the ascending and transverse colon. This feature is very rare in chronic GVHD and resembles the feature in autoimmune disease such as periarteritis nodosa. Thus, this ulceration is thought to be caused by vasculitis due to an autoimmune reaction in chronic GVHD. PMID:7742748
Murayama, T; Nakagawa, T; Matsushita, K; Matozaki, S; Yasutake, K; Kizaki, T; Sashikata, T
Splenic Infarct Due to Scrub Typhus
Scrub typhus is a mite borne infectious disease that has the potential to involve multiple organs and can be fatal. Involvement of the abdomen in the form of hepatitis, gastric ulcerations and pancreatitis are well-documented, the pathology being disseminated vasculitis. However involvement of the spleen in scrub typhus is extremely rare and is reported only in a few autopsy studies. We report the case of a 50-year-old lady who presented with fever and left upper quadrant abdominal pain due to a splenic infarct due to scrub typhus. PMID:24926171
Raj, Selvin Sundar; Krishnamoorthy, Adhiti; Jagannati, Manjeera; Abhilash, Kundavaram PP
Microscopic polyangiitis (MPA) is a necrotizing vasculitis involving the small vessels without granulomatous inflammation. Most MPA initially presents with renal involvement without pulmonary involvement. Isolated and initially presenting alveolar hemorrhage is very rare. The patient was a 39-year-old female with a progressive cough, dyspnea, and blood-tinged sputum for the previous 5 days. We determined that her condition was MPA though VATS lung biopsy and renal biopsy. After 2 months of steroid therapy, the chest lesions had improved. We report here a rare case of MPA with isolated and initial involvement of the lung with a review of the literature. PMID:22324034
Kim, Jae Jun; Wang, Young-Pil; Park, Hyung Joo; Sung, Sook-Whan; Kim, Do-Yeon
Fistulization of rheumatoid joints. Spectrum of identifiable syndromes.
Eight patients with rheumatoid arthritis developed cutaneous fistulae adjacent to affected joints. Rheumatoid factor was positive in eight patients; subcutaneous nodules were noted in seven. Two patients had features of rheumatoid vasculitis. A spectrum of syndrome characterized by cutaneous fistulae was observed. Three patients showed classical fistulous rheumatism. Four patients developed septic arthritis which subsequently fistulized; in two, infection was associated with total joint replacement. One patient showed a cutaneous sinus accompanying a large calf cyst. A variety of diagnoses must be considered when cutaneous fistulae appear near joints in patients with rheumatoid arthritis. Images PMID:1221937
Shapiro, R F; Resnick, D; Castles, J J; D'Ambrosia, R D; Lipscomb, P R
Lymphocytic Thrombophilic Arteritis: An Enigma
A 55-year-old woman presented with a 5-year history of livedo racemosa on her limbs. Histology showed vasculitis of medium-sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymphocytic thrombophilic arteritis (LTA), alias macular arteritis, which is a recently described entity. The importance of LTA lies in the fact that it is a close clinical and microscopic mimic of polyarteritis nodosa (PAN). LTA is believed to be a distinct entity by some and as a form of PAN by others. We have discussed this case in our report.
Kalegowda, Inchara Yeliur; Tirumalae, Rajalakshmi; Murthy, K Srinivasa; Rout, Pritilata
Systemic necrotic and granulomatous vasculitis presents with vascular involvement in almost every organ, manifesting with stenosis, ischemia, infarction, or hemorrhage. The clinical picture depends on the kind and size of the involved vessels and on the activity of the inflammatory process. Wegener's granulomatosis is a necrotic, granulomatous inflammation of small vessels of the upper and lower airways and kidneys. We present the case of a 25-year-old male in whom the definitive diagnosis of Wegener's granulomatosis was achieved within five weeks from onset. Among the first symptoms were microinfarcts localized in the fingertips and the nail matrix; their nature was investigated with capillaroscopy. PMID:21427813
Matuszewska, Agnieszka; Misterska-Sk�ra, Maria; Wiland, Piotr
Behcet's disease and cardiovascular involvement: our experience of asymptomatic Behcet's patients
Summary Abstract Behcet�s syndrome is a systemic inflammatory disease associated with vasculitis, and arterial, venous and cardiac disorders. Thirty-eight Behcet�s disease patients were examined prospectively with echocardiography, ultrasonography and computed tomography, and coagulation parameters were determined. Deep venous insufficiency was found in 16 patients, venous thrombosis in seven, one patient had iliac artery stenosis, three had carotid arterial intimal proliferation, two patients had aortic annulus dilatation, six had aortic valve insufficiency, and three had mitral valve insufficiency. None had coagulation defects. To decrease morbidity and mortality rates, a multidisciplinary approach is important for early diagnosis of cardiovascular involvement in Behcet�s disease. PMID:24844550
Ulusan, Zeynep; Karadag, Ayse Serap; Tasar, Mehmet; Kalender, Mehmet; Tansel Darcin, Osman
A clinicopathological classification of granulomatous disorders
Granulomatous disorders comprise a large family sharing the histological denominator of granuloma formation. A granuloma is a focal compact collection of inflammatory cells, mononuclear cells predominating, usually as a result of the persistence of a non-degradable product and of active cell mediated hypersensitivity. There is a complex interplay between invading organism or prolonged antigenaemia, macrophage activity, a Th1 cell response, B cell overactivity and a vast array of biological mediators. Differential diagnosis and management demand a skilful interpretation of clinical findings and pathological evidence. They are classified into infections, vasculitis, immunological aberration, leucocyte oxidase deficiency, hypersensitivity, chemicals, and neoplasia.???Keywords: granuloma; Th1 cell; cytokines; neoplasia PMID:10908370
James, D
Imaging Characteristics of Takayasu Arteritis
Takayasu arteritis is a rare, large-vessel vasculitis in which the nonspecific systemic inflammatory symptoms are followed by inflammation of the aorta and its major branches. The inflammation of this vessel leads to progressive luminal stenosis or aneurysm formation resulting in limb or organ ischemia. Although conventional angiography is still accepted as the gold standard modality, the information obtained is limited to the vessel lumen. Multidetector computed tomographic angiography and magnetic resonance angiography can provide valuable information not only regarding intraluminal pathologies but also concerning the thickening of the vessel wall, which may be the earliest manifestation of the disease.
Canyigit, Murat, E-mail: email@example.com; Peynircioglu, Bora; Hazirolan, Tuncay; Dagoglu, Merve Gulbiz; Cil, Barbaros Erhan; Haliloglu, Mithat; Balkanci, Ferhun; Besim, Aytekin [Faculty of Medicine, Hacettepe University, Department of Radiology (Turkey)
Repeated surgeries in a patient with Beh?et's disease, what changes to expect?
Beh�et's disease is a multisystem inflammatory disorder of unknown etiology characterized by vasculitis of large and smaller vessels. Most common features are appearance of oral ulcers which later heal by scarring and ocular involvement leading to glaucoma requiring surgical treatment. Here, we report a case of child suffering from Beh�et's disease undergoing repeated ocular surgery for uncontrolled glaucoma and found that with the usual risk involved in such patients there are important changes in the airway when the patient is exposed to repeated surgery under general anesthesia.
Gupta, Anurag; Banerjee, Neerja; Sharma, Jyoti; Sood, Rajesh
Neutrophil serine proteases are specific regulators of the immune response, and proteinase 3 is a major target antigen in antineutrophil cytoplasmic antibody-associated vasculitis. FRET peptides containing 2-aminobenzoic acid (Abz) and N-(2,4-dinitrophenyl)ethylenediamine (EDDnp) as fluorophore and quencher groups, respectively, have been widely used to probe proteases specificity. Using in silico design followed by enzymatic assays, we show that Abz and EDDnp significantly contribute to substrate hydrolysis by PR3. We also propose a new substrate specific for PR3. PMID:24397704
Narawane, Shailesh; Budnjo, Adnan; Grauffel, C�dric; Haug, Bengt Erik; Reuter, Nathalie
Central venous catheter infection-induced Henoch-Sch�nlein purpura in a patient on hemodialysis.
A 69-year-old man, who had been dialyzed using a permanent central venous catheter for 2 years, presented with Henoch-Sch�nlein purpura and positive perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA). He was diagnosed with catheter-related infection by Staphylococcus aureus. After administration of antibiotic and steroid therapy, purpura disappeared and p-ANCA gradually became negative. This case supports the conclusion that infection can be pathogenesis of the vasculitis, including ANCA-positive HSP. Additionally, impregnation of catheters with antibiotics can be an effective treatment for catheter infections. PMID:24845224
Gao, Jian Jun; Wei, Jia Mei; Gao, Yue Hua; Li, Shuang; Na, Yu
The paper presents data on the diagnosis, clinical and pathomorphological changes in the central nervous system (CNS) in neurochlamydiasis according to clinical, autoptic, and experimental evidence. It discusses the possible implication of Ch. trachomatis, Ch. pneumoniae, and Ch. psittaci in the development and course of different diseases with CNS involvement: atherosclerosis, vasculitis, multiple sclerosis, Alzheimer's disease, schizophrenia, autism, vegetative state, sequels of perinatal lesions in childhood and adolescence, HIV infection, etc. Considerable attention is paid to the specific features of diagnosis of Chlamydia-induced CNS lesions. Purposeful pathomorphological investigations are shown to be needed. PMID:24745195
Va?nshenker, Iu I; Nuralova, I V; Onishenko, L S
Zygomycotic necrotizing fasciitis caused by Apophysomyces elegans.
A case of necrotizing fasciitis of the anterior abdominal wall caused by the zygomycete Apophysomyces elegans in a healthy male following inguinal herniorrhaphy is reported. The portal of entry of the fungus into the incised skin and subcutaneous tissues was probably through either contaminated surgical sutures or postoperative surgical dressings. Broad, aseptate fungal hyphae were seen in the necrosed tissues with an associated necrotizing vasculitis. Extensive tissue debridements and a low dose of amphotericin B were not successful in controlling the rapid invasion of the tissues by the fungus. Images PMID:8501244
Lakshmi, V; Rani, T S; Sharma, S; Mohan, V S; Sundaram, C; Rao, R R; Satyanarayana, G
Zygomycotic necrotizing fasciitis caused by Apophysomyces elegans.
A case of necrotizing fasciitis of the anterior abdominal wall caused by the zygomycete Apophysomyces elegans in a healthy male following inguinal herniorrhaphy is reported. The portal of entry of the fungus into the incised skin and subcutaneous tissues was probably through either contaminated surgical sutures or postoperative surgical dressings. Broad, aseptate fungal hyphae were seen in the necrosed tissues with an associated necrotizing vasculitis. Extensive tissue debridements and a low dose of amphotericin B were not successful in controlling the rapid invasion of the tissues by the fungus. PMID:8501244
Lakshmi, V; Rani, T S; Sharma, S; Mohan, V S; Sundaram, C; Rao, R R; Satyanarayana, G
Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity. PMID:22852104
Cernea, Daniela; Dragoescu, Alice; Novac, Marius
Purtscher's retinopathy is usually associated with trauma, acute pancreatitis, vasculitis, lupus, and bone fractures. It was rarely described postpartum in patients with preeclampsia as well as associated with HELLP syndrome. We present a case of a multiparous patient aged 44 with severe preeclampsia and postpartum HELLP syndrome complicated with Purtscher-like retinopathy and large ruptured subcapsular liver hematoma that required emergency abdominal surgery after premature delivery of a dead fetus. Postsurgical outcome was favorable regarding both liver function and visual acuity. PMID:22852104
Cernea, Daniela; Dragoescu, Alice; Novac, Marius
[Beh�et's disease revealed by deep vein thrombosis in an HIV-1-infected patient].
Beh�et's disease is a multisystemic vasculitis characterized by the association of recurrent oral and genital ulcers with systemic involvements, particularly ocular, nervous and vascular manifestations. The association of Beh�et's disease and aids/HIV infection is rare. The role of HIV infection in the pathomechanism of both diseases is unclear. We report a 28-year-old HIV-positive patient who presented Beh�et's disease complicated by deep vein thrombosis and left lateral sinus thrombosis. This rare association raises questions about the etiopathogenic process and therapeutic management. PMID:23068309
Marih, L; Sodqi, M; Marhoum El Filali, K; Chakib, A
[Necrotizing fasciitis after varicella].
Necrotizing fasciitis is a rare and severe infection characterised by extremely rapid progressive involvement of the superficial fascias and deep dermal layers of the skin, with resultant vasculitis and necrosis. The authors present three clinical cases of necrotizing fasciitis; all three patients previously had varicella rash, rapid progressive spreading erythema with severe pain and toxic shock syndrome. Two patients had positive cultures of b-haemolytic streptococcus. Early stage differential diagnosis with celulitis, aggressive antibiotic treatment and pediatric intensive care support are essential. However, the main therapy is early extensive surgical approach involving all indurate areas, down to and including the muscle fascia. PMID:11552336
Gon�alves, E; Furtado, F; Estrada, J; Vale, M C; Pinto, M; Santos, M; Moura, G; Vasconcelos, C
Beh�et's disease (BD) is a multisystemic vasculitis syndrome characterized by a course of remissions and exacerbations of unpredictable frequency and duration. The disease has a worldwide distribution, but the majority of cases cluster along the ancient Silk Road, which extends from eastern Asia to the Mediterranean basin. The etiopathogenesis of BD is still unknown, but familial aggregation and peculiar geographical distribution have been regarded as evidence supporting genetic influence on the pathogenesis of BD. In this article, we describe a patient with BD, who had four members of his family associated with BD. PMID:19575202
Yilmaz, Sema; Cimen, Kadriye Akar
[Neuro-Beh�et in a Caucasian Danish patient].
Beh�et's disease (BD) is a chronic, relapsing multisystem inflammatory disorder, vasculitis being its major pathological feature. It is prevalent in the Orient and areas along the ancient Silk Road, but very rare among North European Caucasians. Neurological involvement is relatively uncommon. The diagnosis of neuro-Beh�et can be difficult to establish if the mucocutaneous involvement is incomplete, absent, or unattended. We present an exceptional case of neuro-Beh�et in a 38-year-old Caucasian Danish woman. On account of the risk of serious complication, it is important to make the diagnosis as soon as possible. PMID:20184824
Karshena, Ali; Koch-Hendriksen, Nils J
Beh�et's disease is a systemic vasculitis of unknown aetiology characteristically affecting venules. Onset is typically in young adults with recurrent oral and genital ulceration, uveitis, skin manifestations, arthritis, neurological involvement, and a tendency to thrombosis. It has a worldwide distribution but is prevalent in Japan, the Middle East, and some Mediterranean countries. International diagnostic criteria have been proposed, however diagnosis can be problematical, particularly if the typical ulcers are not obvious at presentation. Treatment is challenging, must be tailored to the pattern of organ involvement for each patient and often requires combination therapies.???Keywords: Beh�et's disease; oral ulcers; uveitis; immunosuppressants PMID:11009577
Kontogiannis, V; Powell, R
We describe 3 unusual cases of granuloma annulare with multiple macular lesions in a distribution that simulated mycosis fungoides in patients with no associated underlying diseases. Repeated biopsies showed typical well-formed palisading granulomas and no evidence of an atypical lymphocytic infiltrate. There was no vasculitis, neutrophilic, eosinophilic, or interstitial infiltrate. The patients had no associated underlying diseases. Most of the histiocytes in the palisading granulomas were strongly positive for CD68. The lymphocytes were a minor component of the granulomatous inflammation and were predominantly CD8(+) T-cells. The findings in these cases add to the spectrum of previously defined granulomatous eruptions of the skin. PMID:15243522
Wu, Hong; Barusevicius, Alan; Lessin, Stuart R
Malignant catarrhal fever associated with ovine herpesvirus-2 in free-ranging mule deer in Colorado.
Malignant catarrhal fever (MCF) was diagnosed in four free-ranging mule deer (Odocoileus hemionus) in January and February of 2003. Diagnosis was based on typical histologic lesions of lymphocytic vasculitis and PCR identification of ovine herpesvirus-2 (OHV-2) viral genetic sequences in formalin-fixed tissues. The animals were from the Uncompahgre Plateau of southwestern Colorado. Deer from these herds occasionally resided in close proximity to domestic sheep (Ovis aries), the reservoir host of OHV-2, in agricultural valleys adjacent to their winter range. These cases indicate that fatal OHV-2 associated MCF can occur in free-ranging mule deer exposed to domestic sheep that overlap their range. PMID:17699095
Schultheiss, Patricia C; Van Campen, Hana; Spraker, Terry R; Bishop, Chad; Wolfe, Lisa; Podell, Brendan
Odocoileus hemionus deer adenovirus is related to the members of Atadenovirus genus
Summary.��?The Odocoileus hemionus deer adenovirus (OdAdV-1) causes systemic and local vasculitis and proves extremely lethal for mule deer. To characterize\\u000a the virus, part of the genome flanking the fiber gene was cloned and sequenced. The sequence revealed two open-reading frames\\u000a that mapped to pVIII hexon-associated protein precursor and fiber protein of several other adenoviruses. The highest amino\\u000a acid homology for
A. Zakhartchouk; A. Bout; L. W. Woods; H. D. Lehmkuhl; M. J. E. Havenga
Giant Cell Arteritis in a 12-Year-Old Girl Presenting with Nephrotic Syndrome
Giant cell arteritis (GCA) is rare in children. The kidneys are generally spared. We present a case of GCA in a 12-year-old girl with severe headache and tender scalp especially over the right temporal area. The right superficial temporal artery was cord like and nodular and the pulsations were barely felt. Several small tender nodular swellings were felt in the occipital area. She had been previously diagnosed as a case of nephrotic syndrome due to underlying membranoproliferative glomerulonephritis. This report is aimed at drawing attention to this rare form of vasculitis in children aiming at decreasing its morbidities.
El-Sayed, Zeinab A.; El-Awady, Hanaa M.; Hassan, Zeinab E.; Adham, Tamer M. H.; Mostafa, Hossam M.; Elhefnawy, Nadia G.
Role of neutrophils in murine cryoglobulinemia
Murine IgG3 anti-IgG2a rheumatoid factor (RF) monoclonal antibodies (mAb) with cryoglobulin activity, are able to induce, in normal mice, skin leukocytoclastic vasculitis and lupus-like glomerulonephritis resembling 'wire-loop' lesions (subendothelial immune deposits). The development of glomerular, but not skin, lesions in immunoglobulin-deficient mice (lacking the corresponding IgG2a autoantigen) receiving IgG3 RF cryoglobulins indicates that the RF activity of IgG3 monoclonal cryoglobulins
S. Izui; T. Fulpius; L. Reininger; Y. Pastore; T. Kobayakawa
A young patient with history of Kawasaki disease presenting with triple vessel disease.
Kawasaki disease is primarily a condition that affects young children and it is associated with cardiac morbidity and mortality. This disease has been known to cause coronary artery aneurysms which occurs as a sequelae of vasculitis. The progression of triple vessel disease in adult which results from cardiac complications from Kawasaki disease is rare. We report a case of a young man with history of Kawasaki disease at infancy presenting with triple vessel disease requiring cardiac bypass surgery at the age of 20 years old. PMID:22822650
Lim, A L; Lam, H Y; Kareem, B A; Kamarulzaman, M H
Leprosy with Guillain Barre Syndrome: a new neurologic manifestation?
A 19-year-old female patient of lepromatous leprosy with Type II reaction, on multidrug therapy and prednisolone, presented with acute onset flaccid quadriparesis. The cerebrospinal fluid examination revealed albumino-cytologic dissociation. Nerve biopsy showed infiltration with lepra bacilli, features of vasculitis, and demyelination. There were no other identifiable precipitating factors for Guillain Barre Syndrome in this patient. Her condition improved without any steroid therapy. This case emphasizes the hypothesis that cell injury caused by Type II reaction can expose neural antigens and incite an autoimmune reaction in the form of Guillain Barre Syndrome. PMID:15160867
Grover, Chander; Kubba, Samir; Nanda, Soni; Kumar, Vijay; Reddy, Belum Siva Nagi
Hepatitis C-associated rheumatic disorders.
Hepatitis C virus (HCV) is an important causative agent of liver diseases. However, HCV infection is also associated with numerous hematologic, renal, dermatologic, rheumatic, and autoimmune disorders. These include arthralgia, arthritis, vasculitis, sicca syndrome, myalgia, and fibromyalgia. The purpose of this article is to review the prevalence and spectrum of rheumatic disorders and autoimmune phenomena in HCV-infected patients. It evaluates and current treatment options including nonsteroidal anti-inflammatory drugs, low-dose corticosteroids, hydroxychloroquine, methotrexate, penicillamine, combined antiviral therapy, cyclosporin A, anti-TNF-a agents, and rituximab. It concludes that larger, controlled studies are needed to establish further the treatment indications, efficacy, and safety of these agents. PMID:19481000
Buskila, Dan
Extracorporeal removal of circulating immune complexes: from non-selective to patient-specific.
The classical immune complex-mediated disease, termed serum sickness, developed a short time after the injection of horse anti-tetanus toxin. Antibodies against circulating horse plasma proteins lead to the formation of immune complexes within the blood circulation (CIC). The inflammatory response, including systemic complement activation and vasculitis, seriously affected the function of all organs, including the most susceptible kidney. Meanwhile CIC have been detected in almost every systemic disease, including autoimmune disorders and also cancer and infections. This brief review will focus on the rationale and the equipment for extracorporeal elimination of CIC. PMID:10838476
Matic, G; Sch�tt, W; Winkler, R E; Tiess, M; Ramlow, W
How anti-neutrophil cytoplasmic autoantibodies activate neutrophils.
Neutrophils are pivotal to host defence during infectious diseases. However, activated neutrophils may also cause undesired tissue damage. Ample examples include small-vessel inflammatory diseases (vasculitis) that are associated with anti-neutrophil cytoplasmic autoantibodies (ANCA) residing in the patients' plasma. In addition to being an important diagnostic tool, convincing evidence shows that ANCA are pathogenic. ANCA-neutrophil interactions induce important cellular responses that result in highly inflammatory necrotizing vascular damage. The interaction begins with ANCA binding to their target antigens on primed neutrophils, proceeds by recruiting transmembrane molecules to initiate intracellular signal transduction and culminates in activation of effector functions that ultimately mediate the tissue damage. PMID:22861361
Kettritz, R
Lymphocytic thrombophilic arteritis: an enigma.
A 55-year-old woman presented with a 5-year history of livedo racemosa on her limbs. Histology showed vasculitis of medium-sized arteries with a circumferential, hyalinised, intraluminal fibrin ring. Her laboratory investigations did not indicate any underlying systemic disease. The findings were consistent with lymphocytic thrombophilic arteritis (LTA), alias macular arteritis, which is a recently described entity. The importance of LTA lies in the fact that it is a close clinical and microscopic mimic of polyarteritis nodosa (PAN). LTA is believed to be a distinct entity by some and as a form of PAN by others. We have discussed this case in our report. PMID:25284860
Kalegowda, Inchara Yeliur; Tirumalae, Rajalakshmi; Murthy, K Srinivasa; Rout, Pritilata
Behcet's disease is a complex multisystemic chronic inflammatory disease that is characterized by oral and genital aphtous ulcers and vasculitis. Aneurysms of major arteries are the most important cause of mortality in Behcet's disease. Four patients with superior mesenteric artery (SMA) aneurysms related to Behcet's disease have been reported in the literature. We report here the first successful endovascular treatment of a giant, wide-necked SMA aneurysm secondary to Behcet's disease. We performed a balloon-assisted embolization technique using ethylene vinyl alcohol copolymer (Onyx, ev3, Irvine, CA, USA). There were no signs of recurrence during 2-year follow-up.
Gueven, Koray, E-mail: email@example.com; Rozanes, Izzet, E-mail: firstname.lastname@example.com [Istanbul University, Department of Radiology, Istanbul Faculty of Medicine (Turkey); Kayabali, Murat, E-mail: firstname.lastname@example.com [Istanbul University, Section of Vascular Surgery, Department of General Surgery, Istanbul Faculty of Medicine (Turkey); Minareci, Ozenc, E-mail: firstname.lastname@example.com [Istanbul University, Department of Radiology, Istanbul Faculty of Medicine (Turkey)
Comparative study of group A and group C meningococcal infection.
114 patients with meningococcal infection were studied; 72 had group C infection and 42 group A infection. 14 patients had acute meningococcaemia, all of whom had group C infection and 9 of whom died. Clinical and laboratory findings were similar in patients with meningitis due to a group A and C organisms, but arthritis and cutaneous vasculitis were more common in patients with group C infection. The overall mortality was 22% in patients with group C infection, and 12% in patients with group A infection, but was the same in both groups when cases of acute meningococcaemia are excluded. PMID:860875
Evans-Jones, L G; Whittle, H C; Onyewotu, I I; Egler, L J; Greenwood, B M
Clinical presentation of Churg-Strauss syndrome in children
Churg�Strauss syndrome is an uncommon multisystem disorder characterized by asthma, eosinophilia and vasculitis. We report on a 12-year-old boy with asthma and deterioration of his general condition, who was eventually diagnosed with an ANCA-negative Churg�Strauss syndrome. The propositus included, 50 cases of childhood Churg�Strauss syndrome have been reported. The patient characteristics and clinical characteristics of these children are summarized. The respiratory tract is most frequently involved with pulmonary infiltrates, asthma and sinusitis. Early recognition of childhood Churg�Strauss syndrome is important as delayed diagnosis can lead to severe organ involvement, and possible fatal outcome.
Razenberg, Femke G.E.M.; Heynens, Jan W.C.M.; Jan de Vries, Geeuwke; Duijts, Liesbeth; de Jongste, Johan C.; de Blic, Jacques; Rosias, Philippe P.R.
Giant aortic arch aneurysm complicating Kawasaki's disease
Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small- and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies, related reports on peripheral arterial and aortic aneurysms are scarce. We report the occurrence of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28-month-old boy diagnosed with KD. This complication was managed by steroids therapy in the beginning. Because of mechanical complication and potential risk of rupture, surgery was undertaken.
Hakim, Kaouthar; Boussada, Rafik; Chaker, Lilia; Ouarda, Fatma
Generalized vasculitic exanthem following Loxosceles reclusa envenomation.
Brown recluse spider bites (BRSB) cause a myriad of reactions ranging from local necrosis to potentially lethal systemic involvement. Envenomation may induce a generalized exanthem known clinically but not described histologically. We report a 49-year-old female who developed a generalized exanthem 24 hours after BRSB. The histopathology demonstrated a necrotizing vasculitis similar to that seen at an envenomation site but without epidermal necrosis. Loxoscelism should be considered in patients residing in endemic areas who present with a vasculitic exanthem. PMID:17518783
Robb, Christopher W; Hayes, Benjamin B; Boyd, Alan S
Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature
About one-third of patients suffering from systemic lupus erythematosus have ocular manifestations. The most common manifestation is keratoconjunctivitis sicca. The most vision threatening are retinal vasculitis and optic neuritis/neuropathy. Prompt diagnosis and treatment of eye disease is paramount as they are often associated with high levels of systemic inflammation and end-organ damage. Initial management with high-dose oral or IV corticosteroids is often necessary. Multiple �steroid-sparing� treatment options exist with the most recently studied being biologic agents. PMID:22811887
Palejwala, Neal V.; Walia, Harpreet S.; Yeh, Steven
Grand Rounds: Latex-Induced Occupational Asthma in a Surgical Pathologist
Context: Latex allergy and sensitization have been an important problem facing health care workers. Providing a latex-safe environment is the intervention of choice. Case Presentation: A 46-year-old surgical pathologist presented with increasing shortness of breath for the previous 4 years. Twenty years before presentation, he noted a pruritic, erythematous rash on his hands, associated with latex glove use. Fourteen years before presentation, during pathology residency, he developed a nonproductive cough, wheezing, and an urticarial rash, temporally associated with use of powdered latex gloves. These symptoms improved while away from work. At presentation, he had one-flight dyspnea. His skin prick test was positive for latex, and pulmonary function testing showed mild obstruction, which was reversible with bronchodilator use. Because the patient was at risk for worsening pulmonary function and possible anaphylaxis with continued exposure, he was removed from the workplace because no reasonable accommodation was made for him at that time. Discussion: The patient�s presentation is consistent with latex-induced occupational asthma. Initially noting dermal manifestations, consistent with an allergic contact dermatitis secondary to accelerators present in latex gloves, he later developed urticaria, flushing, and respiratory symptoms, consistent with a type I hypersensitivity reaction to latex. He also has reversible airways disease, with significant improvement of peak expiratory flow rate and symptoms when away from work. Relevance to Clinical or Professional Practice: The ideal treatment for latex sensitization is removal from and avoidance of exposure. Clinicians should consider occupational asthma when patients present with new-onset asthma or asthmatic symptoms that worsen at work. PMID:16002378
Green-McKenzie, Judith; Hudes, Debra
Neonatal-Onset Multisystem Inflammatory Disease Responsive to Interleukin-1? Inhibition
BACKGROUND Neonatal-onset multisystem inflammatory disease is characterized by fever, urticarial rash, aseptic meningitis, deforming arthropathy, hearing loss, and mental retardation. Many patients have mutations in the cold-induced autoinflammatory syndrome 1 (CIAS1) gene, encoding cryopyrin, a protein that regulates inflammation. METHODS We selected 18 patients with neonatal-onset multisystem inflammatory disease (12 with identifiable CIAS1 mutations) to receive anakinra, an interleukin-1�receptor antagonist (1 to 2 mg per kilogram of body weight per day subcutaneously). In 11 patients, anakinra was withdrawn at three months until a flare occurred. The primary end points included changes in scores in a daily diary of symptoms, serum levels of amyloid A and C-reactive protein, and the erythrocyte sedimentation rate from baseline to month 3 and from month 3 until a disease flare. RESULTS All 18 patients had a rapid response to anakinra, with disappearance of rash. Diary scores improved (P<0.001) and serum amyloid A (from a median of 174 mg to 8 mg per liter), C-reactive protein (from a median of 5.29 mg to 0.34 mg per deciliter), and the erythrocyte sedimentation rate decreased at month 3 (all P<0.001), and remained low at month 6. Magnetic resonance imaging showed improvement in cochlear and leptomeningeal lesions as compared with baseline. Withdrawal of anakinra uniformly resulted in relapse within days; retreatment led to rapid improvement. There were no drug-related serious adverse events. CONCLUSIONS Daily injections of anakinra markedly improved clinical and laboratory manifestations in patients with neonatal-onset multisystem inflammatory disease, with or without CIAS1 mutations. (ClinicalTrials.gov number, NCT00069329.) PMID:16899778
Goldbach-Mansky, Raphaela; Dailey, Natalie J.; Canna, Scott W.; Gelabert, Ana; Jones, Janet; Rubin, Benjamin I.; Kim, H. Jeffrey; Brewer, Carmen; Zalewski, Christopher; Wiggs, Edythe; Hill, Suvimol; Turner, Maria L.; Karp, Barbara I.; Aksentijevich, Ivona; Pucino, Frank; Penzak, Scott R.; Haverkamp, Margje H.; Stein, Leonard; Adams, Barbara S.; Moore, Terry L.; Fuhlbrigge, Robert C.; Shaham, Bracha; Jarvis, James N.; O'Neil, Kathleen; Vehe, Richard K.; Beitz, Laurie O.; Gardner, Gregory; Hannan, William P.; Warren, Robert W.; Horn, William; Cole, Joe L.; Paul, Scott M.; Hawkins, Philip N.; Pham, Tuyet Hang; Snyder, Christopher; Wesley, Robert A.; Hoffmann, Steven C.; Holland, Steven M.; Butman, John A.; Kastner, Daniel L.
Therapeutic apheresis in the Republic of Macedonia - our five years experience (2000-2004).
Membrane plasma exchange (PE) is a mode of extracorporeal blood purification. Since 1985 membrane PE has been in regular use at the Department of Nephrology, Medical Faculty of Skopje, R.Macedonia. In this paper we report on five years (2000-2004) of single centre plasma exchange activity. We performed 540 PE treatments (108 PE/per year) on 99 patients. The M/F ratio was 40/48. The patients underwent a median of 5.45 procedures (range, 1-16). The treated patients were from different Departments. Protocols for PE depend on the disease and its severity. PE were performed 2-4 times weekly using Gambro PF 2000 N filters with an adaptation of the Gambro AK10 dialysis machine or with the Gambro Prizma machine (2 cases). Blood access was achieved through femoral vein. Substitution was made with fresh frozen plasma and/or with 20% human albumin combined with Ringer's solution. An average amount of 2150 ml plasmafiltrate per treatment (respectively 30 to 40 ml plasmafiltrate/kg body weight) was eliminated. Most therapeutic procedures were performed on patients from the Department of Neurology. 63.6% of all patients were referred for Myasthenia gravis and the Guillian Barre syndrome. The total number of procedures per year has remained fairly stable, corresponding to a median of 5.4 treatments/100 000 inhabitants. We observed hypocalcaemia in 8% of the patients, urticarial reactions in 7.3%, pruritic reactions in 12%, and hypotension/headache in 6.8%. No major procedural complications were seen. PMID:16862064
Milovanceva-Popovska, M; Stojkovski, Lj; Grcevska, L; Dzikova, S; Ristovska, V; Gogovska, L; Polenakovic, M
The autoinflammatory diseases.
The monogenic autoinflammatory syndromes are conditions caused by mutations of genes coding for proteins that play a pivotal role in the regulation of the inflammatory response. Due to their genetic nature, most of these disorders have an early onset. Clinically they are characterised by recurrent flares of systemic inflammation presenting most of the time as sudden fever episodes associated with elevation of acute phase reactants and with a number of clinical manifestations such as rash, serositis, lymphadenopathy and arthritis. Symptom-free intervals are characterised by complete wellbeing, normal growth and complete normalisation of acute phase reactants. Familial Mediterranean fever (FMF), mevalonate-kinase deficiency (MKD) and tumour necrosis factor (TNF) receptor-associated periodic syndrome (TRAPS) are the three monogenic disorders subsumed under the term periodic fevers, while a systemic inflammation dominated by a characteristic urticarial rash associated with a number of other clinical manifestations is typical of familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and chronic infantile neurological cutaneous and articular syndrome (CINCA). These diseases represent the clinical spectrum of different mutations of a gene named cold-induced autoinflammatory syndrome 1 (CIAS-1, or NLRP3) coding for a protein called cryopyrin. Hence these disorders are also known as cryopyrin-associated periodic syndromes (CAPS). Other conditions are characterised by typical granulomatous formations (granulomatous disorders). Blau's syndrome (familial juvenile systemic granulomatosis) presents with non-caseating granulomatous inflammation affecting the joint, skin, and uveal tract (the triad of arthritis, dermatitis and uveitis) and is associated with mutations of the NACHT domain of the gene CARD15 (or NOD2). PMID:22714396
Federici, Silvia; Caorsi, Roberta; Gattorno, Marco
Urticaria--an allergologic, dermatologic or multidisciplinary disease?
Urticaria is a frequent disease, with complex etiopathogeny, raising important problems in clinical practice. The life-time prevalence for any subtype of urticaria is about 20%. Urticaria and/or angioedema is a heterogeneous group of diseases that result from a large variety of underlining causes, are elicited by a great diversity of factors and present clinically in a high variable way. In the past few decades an increasing understanding of the pathomechanisms involved in urticaria has highlighted the heterogeneity of different subtypes. According to the clinical picture and associated signs and symptoms, urticaria can be a simple, self-limited disease or a very complicated and debilitating one. Urticaria is frequently caused by allergic reactions, but there are also many nonallergic causes. The majority cases of chronic urticaria have unknown (idiopathic) causes, with about 30-40% possibly having an autoimmune substrate. An autoimmune subset of chronic spontaneous urticaria is increasingly being recognized internationally, based on laboratory and clinical evidence that accrued over the last 20 years. Urticarial rash may be part of clinical picture of autoinflammatory diseases, a group of rare disorders of the innate immune system, mediated by Interleukin-1. Since urticaria is an early and prominent symptom of a complex clinical picture, the awareness of these disorders may help the diagnostic in early stages and prevent severe long-term complications. Management of urticaria is based on recommendations of EAACI/GA2LEN/EDF/WAO Guideline published in 2009 and updated during the 4th International Consensus Meeting on Urticaria (Berlin, November 2012). PMID:24620623
Leru, Poliana
Consequences of captivity: health effects of far East imprisonment in World War II.
Though medical consequences of war attract attention, the health consequences of the prisoner-of-war (POW) experience are poorly researched and appreciated. The imprisonment of Allied military personnel by the Japanese during the World War II provides an especially dramatic POW scenario in terms of deprivation, malnutrition and exposure to tropical diseases. Though predominantly British, these POWs also included troops from Australia, Holland and North America. Imprisonment took place in various locations in Southeast Asia and the Far East for a 3.5-year period between 1942 and 1945. Nutritional deficiency syndromes, dysentery, malaria, tropical ulcers and cholera were major health problems; and supplies of drugs and medical equipment were scarce. There have been limited mortality studies on ex-Far East prisoners (FEPOWs) since repatriation, but these suggest an early (up to 10 years post-release) excess mortality due to tuberculosis, suicides and cirrhosis (probably related to hepatitis B exposure during imprisonment). In terms of morbidity, the commonest has been a psychiatric syndrome which would now be recognized as post-traumatic stress disorder--present in at least one-third of FEPOWs and frequently presenting decades later. Peptic ulceration, osteoarthritis and hearing impairment also appear to occur more frequently. In addition, certain tropical diseases have persisted in these survivors--notably infections with the nematode worm Strongyloides stercoralis. Studies 30 years or more after release have shown overall infection rates of 15%. Chronic strongyloidiasis of this type frequently causes a linear urticarial 'larva currens' rash, but can potentially lead to fatal hyperinfection if immunity is suppressed. Finally, about 5% of FEPOW survivors have chronic nutritional neuropathic syndromes--usually optic atrophy or sensory peripheral neuropathy (often painful). The World War II FEPOW experience was a unique, though often tragic, accidental experiment into the longer term effects of under nutrition and untreated exotic disease. Investigation of the survivors has provided unique insights into the medical outcome of deprivation in tropical environments. PMID:18854350
Robson, D; Welch, E; Beeching, N J; Gill, G V
Occupational allergic contact dermatitis caused by wood dusts.
Exposure to wood dusts may cause various skin and mucosal symptoms. Allergic dermatoses, caused by wood dusts, diagnosed at the Finnish Institute of Occupational Health during 1976-1999 are reported here. 16 had allergic contact dermatitis and, 2 had contact urticaria. 9 men (3 cabinet makers, 3 joiners, 1 carpenter, 1 knifemaker and 1 machinist) were mainly exposed to tropical hardwoods. 1 man had dermatitis caused by western red cedar. 5 patients, 3 men and 2 women, were exposed to Finnish pine or spruce dusts, and 1 man to aspen. 7 also had rhinitis, 4 asthma or dyspnoea and 3 conjunctivitis. On patch testing, 10 men reacted to 9 different wood dusts, including teak (5), palisander (3), jacaranda (2), mahogany (2), walnut (2) and obeche (1). Reactions to wood allergens, including lapachol (2), deoxylapachol (1), (R)-3,4-dimethoxydahlbergione (2), 2,6-dimethoxy-1,4-benzoquinone (1), mansonone A (2) and salicyl alcohol (1), were noted in 4 cases. All but 1 of 5 patients exposed to pine or spruce dusts reacted to the sawdusts, all 5 to colophonium, 3 to abietic acid, 2 to tall oil resin, 3 to wood tar mix and 4 to other wood gum resins. Of the 2 CU patients, 1 was prick and RAST positive to obeche, 1 reacted with urticarial dermatitis to punah wood dust on chamber exposure. Occupational allergic dermatoses are mainly caused by the dusts of hardwoods, mostly due to Type IV allergy, but may also be caused by softwood dusts. Patch tests can be done with wood dusts, but should be confirmed by patch testing with wood allergens if possible. PMID:11260236
Estlander, T; Jolanki, R; Alanko, K; Kanerva, L
Rituximab for refractory granulomatous eye disease
Objective To determine the effectiveness of rituximab therapy for patients with granulomatous disease of the eye. Methods Retrospective review was undertaken of cases seen at a single institution for ocular antineutrophil cytoplasmic antibody-associated vasculitis or sarcoidosis with persistent ocular disease despite systemic therapy. All patients were treated with rituximab and followed for at least 6 months. Results Nine patients were identified (five with antineutrophil cytoplasmic antibody-associated vasculitis, four with sarcoidosis), and all were treated for at least 6 months. Eight experienced improvement of eye disease and were able to reduce prednisone and other drug therapies. One patient remained stable, but still required high dosages of prednisone. All five patients with lung disease improved with rituximab therapy. Rituximab treatment was well tolerated. Two patients discontinued the drug due to leukopenia; however, both patients reinstituted rituximab at modified doses. Conclusion Rituximab therapy was effective in controlling granulomatous ocular disease in most cases. The drug was corticosteroid-sparing and effective in refractory cases, with no severe adverse events encountered. PMID:23055686
Lower, Elyse E; Baughman, Robert P; Kaufman, Adam H
Lupus Enteritis as an Initial Presentation of Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is an autoimmune disorder which can affect multiple organs and clinical presentation is often a myriad of symptoms; therefore, the index of suspicion should rise when evaluating patients with multiorgan symptomatology. Lupus enteritis is a distinct subset of SLE, defined as either vasculitis or inflammation of the small bowel, with supportive image and/or biopsy findings. The clinical picture of lupus enteritis is often nonspecific, with mild to severe abdominal pain, diarrhea, and vomiting being the cardinal manifestations. Although considered a form of visceral or serosal vasculitis, lupus enteritis is seldom confirmed on histology, making computerized tomography (CT) the gold standard for diagnosis. Lupus enteritis is generally steroid-responsive, and the route of administration is based on clinical status and organ involvement, with preference for intravenous (IV) route in flares with significant tissue edema. The following case describes a young woman presenting with lupus enteritis and lupus panniculitis as an initial manifestation of SLE, the utilization of abdominal CT in diagnosis, and current treatment protocols used for lupus enteritis. PMID:25295199
Sran, Manpreet; Patel, Narmisha
Sclerosing Cholangitis in Beh?et's Disease
Introduction. Sclerosing cholangitis is characterized by an inflammatory and fibrotic lesion of intra- and/or extrahepatic bile ducts. When a causal mechanism of a bile duct lesion is identified, the sclerosing cholangitis is considered secondary. The vasculitis, including the Beh�et disease, is cited as a probable cause of the ischemia and the sclerosing cholangitis. No cases of extrahepatic secondary sclerosing cholangitis have been reported to date. Case Report. We report the first case of secondary sclerosing cholangitis of the extrahepatic bile ducts associated with Beh�et disease in a male who is aged 43, with a previous history of the angio-Beh�et followed by complications of thrombophlebitis and a cerebral thrombophlebitis, and who has a cholestatic jaundice. The diagnosis has been carried out by the MR cholangiopancreatography which has objectified a moderate distension of the intrahepatic bile ducts upstream of regular stacked parietal thickening of the main bile duct. The patient has been treated successfully with the ursodeoxycholic acid and the placement of a plastic stent. Conclusion. This diagnosis should be mentioned to any patient with vasculitis and who has a cholestatic jaundice. PMID:24198838
Ben Slama Trabelsi, Aida; Issaoui, Dhilel; Ksiaa, Mehdi; Souguir, Ahlem; Mama, Nadia; Brahem, Ahlem; Jmaa, Ali; Ajmi, Salem
Rheumatologic manifestations of pediatric HIV infection.
In order to assess the frequency of rheumatologic manifestations at different stages of pediatric human immunodeficiency virus (HIV) infection, 26 HIV-infected children at any stage of infection, seen at the Children's AIDS Clinic of "La Raza" National Medical Center from January 1997 to December 1998, were studied. Rheumatologic manifestations were assessed following the criteria established by the American College of Rheumatology. Blood samples were taken for measuring CD4+ and CD8+ T cells, antinuclear antibodies (ANA), anticardiolipin (ACL) antibodies, and rheumatoid factor (RF). The results were compared to those of 25 HIV-negative children of similar ages. Rheumatologic manifestations were identified in 5 (19.2%) of 26 children. Two of whom were twin sisters with biphasic Raynaud's syndrome, and one had necrosing vasculitis of a finger, as well as lip necrosis and livedo reticularis. These patients were positive for ANA and ACL. One case each of knee arthalgias, vasculitis, and septic arthritis of the ankle were also seen. All of the rheumatologic manifestations were in advanced stages of HIV disease. These rheumatologic changes are similar to those reported for HIV-positive adults, and should be considered as part of the HIV acquired immune deficiency syndrome (AIDS) clinical spectrum in the pediatric population. PMID:11689139
Mart�nez-Rojano, H; Ju�rez Hern�ndez, E; Ladr�n De Guevara, G; del Carmen Gorbea-Robles, M
Adverse immunologic effects of antithyroid drugs.
Propylthiouracil and methimazole are frequently used in the management of hyperthyroidism. Two patients in whom adverse immunologic effects other than isolated agranulocytosis developed during treatment with propylthiouracil are described. A review of the literature revealed 53 similar cases over a 35-year period. Rash, fever, arthralgias and granulocytopenia were the most common manifestations. Vasculitis, particularly with cutaneous manifestations, occurs and may be fatal. The clinical evidence suggests that an immunologic mechanism is involved. A number of different autoantibodies were reported, but antinuclear antibodies were infrequent, and none of the cases met the criteria for a diagnosis of systemic lupus erythematosus. Thus, the reactions do not represent a true drug-induced lupus syndrome. Current hypotheses and experimental data regarding the cause of the reactions are reviewed. No specific clinical subgroup at high risk can be identified, and manifestations may occur at any dosage and at any time during therapy. Cross-reactivity between the two antithyroid drugs can be expected. Except for minor symptoms (e.g., mild arthralgias or transient rash), such reactions are an indication for withdrawal of the drug and the use of alternative methods to control the hyperthyroidism. In rare cases of severe vasculitis a short course of high-dose glucocorticoid therapy may be helpful. PMID:3539299
Wing, S S; Fantus, I G
[A case of miliary tuberculosis showing diffuse alveolar hemorrhage].
A 43-year-old diabetic man had a productive cough and high fever and was admitted to another hospital. His condition did not improve despite treatment with Cefepime, and he was transferred to our hospital. Chest X-ray films and CT findings showed pulmonary infiltration and diffuse ground-glass opacities in bilateral lung fields, but disseminated nodules were not identified. Since his bronchial lavage fluid (BALF) was bloody, we suspected diffuse alveolar hemorrhage due to vasculitis. Steroid pulse therapy was given, and his fever and chest X-ray findings completely improved. However, 1 week later, he again suffered a high fever and bloody sputum, and a chest X-ray film showed granular shadows in bilateral lung fields. He died of respiratory failure on the 18th hospital day despite treatment and mechanical ventilation. An autopsy revealed many necrotizing epithelioid granulomas in both lungs, the liver, the spleen, both kidneys and both adrenal glands. These findings indicated miliary tuberculosis, and a culture of his sputum and BALF finally revealed mycobacterium tuberculosis. Marked alveolar hemorrhage and a hyaline membrane were also found in both lungs, but vasculitis was not recognized in any organ. We report this case, because to the best of our knowledge diffuse alveolar hemorrhage has not been reported as the primary symptom of miliary tuberculosis. PMID:21842695
Nakamura, Sukeyuki; Kamioka, Eiko; Tokuda, Atsuko; Tabeta, Hiroshi
Churg-Strauss syndrome and persistent heart failure: active disease or damage?
Churg-Strauss syndrome (CSS) is a rare small-vessel vasculitis typically associated with adult-onset asthma, peripheral and tissue hypereosinophilia, migratory pulmonary infiltrates, upper respiratory tract symptoms, and clinical evidence of systemic vasculitis. Cardiac involvement is a well-recognized complication with an estimated prevalence of 60%. Heart disease is associated with poor prognosis, accounting for almost 50% mortality in CSS. We present a case of a 48-year-old woman with CSS complicated by congestive heart failure with left ventricular ejection fraction of 25%, who was initially treated with long course of high-dose steroids without any clinical or echocardiographic improvement. She was referred to our hospital 1 year later and was initiated with cyclophosphamide 2 mg/kg per day and prednisone 60 mg/d followed by slow taper. Subsequently, the patient had remarkable improvement. Patient was then transitioned to azathioprine for 1.5 years with sustained disease remission. It may be difficult to determine myocardial disease activity status versus tissue damage in CSS with prolonged duration of heart failure symptoms. This is the first case report demonstrating that CSS cardiac disease may remain active despite 1 year of corticosteroid therapy, and significant improvement or remission can still be achieved by administering more aggressive cytotoxic immunosuppressive therapy. PMID:24048112
Lin, Yih Chang; Oliveira, Guilherme H M; Villa-Forte, Alexandra
Patients with cardiovascular symptoms are mainly diagnosed in cardiological wards. However, sometimes the other reasons for acute coronary syndrome and heart failure are found. One of such reasons is hypereosinophilia which can be recognized if number of blood eosinophils exceeds 1500/mm3. High eosinophilia is connected with production of cytotoxic eosinophilic proteins which can cause eosinophilic vasculitis or eosinophilic myocarditis. One of the better known hypereosinophilic syndromes is EGPA described by the pathomorphologists Churg and Strauss. The further research works allowed for the clinical characteristics of patients with EGPA. In the course of this disease the following three phases were recognized : prodromal-allergic, eosinophilic, vasculitic. The definitive diagnosis can be established only in the third phase, when vasculitis causes organ involvement. Besides symptoms of the respiratory tract (asthma, nasal polyps, eosinophilic lung infiltrations) also cardiovascular symptoms, gastrointestinal tract symptoms, as well as skin lesions and kidneys involvement can appear. The most dangerous for patients is involvement of the nervous and cardiovascular systems. We present a patient with asthma and eosinophilia in whom EGPA was diagnosed in the course of acute recurrent substernal chest pain, with subsequent signs of cardiac insufficiency. PMID:25339570
Za??ska, Jolanta; Wiatr, El?bieta; Zych, Jacek; Szopi?ski, Janusz; Oniszh, Karina; Kober, Jaros?aw; Piotrowska-Kownacka, Dorota; Roszkowski-?li?, Kazimierz
[Ultrasound diagnostic techniques in dermatologic angiology and phlebology].
The diagnostic uses of high-resolution ultrasonography (7.5-20 MHZ) have dramatically advanced in the past 20 years. We present representative examples of ultrasound diagnosis, based on our experience with over 15,000 examinations. For example, peripheral arterial disease was identified in 30% of older patients with venous diseases, while peripheral emboli and vasculitis can lead to widespread skin disease. Ultrasonographic diagnosis is required for appropriate interdisciplinary treatment. In addition, regular evaluation of the supra-aortic vessels and coronary arteries as well as arteries and veins of the limbs is needed in some forms of vasculitis. The causes of peripheral venous disease can be subdivided into four groups based on sonographic findings: Primary or secondary epi- and/or subfascial venous insufficiency, thrombotic venous occlusion, venous compression syndrome and arteriovenous-lymphatic malformations. The diagnosis and therapeutic approach to lymphedema and soft tissue diseases, with or without lymphadenopathy, requires careful sonographic evaluation. Ultrasonography is an essential part of dermatologic oncology, both for planning primary therapy and for follow-up. A sound command of ultrasonography is today an essential aspect of dermatologic diagnosis and therapy. PMID:12835863
Hesse, G; Stiegler, H
Clinical aspects of posterior uveitis in ocular sarcoidosis.
Two clinical forms of the "white spot" syndrome in patients with posterior uveitis in definitive and presumable ocular sarcoidosis were analyzed. Group 1 was characterized by periphlebitis and discrete white spots around the vein of the retina, so-called "candle-wax", whereas group 2 showed yellow-orange solitary nodules located at the choroid, i.e. multifocal choroiditis. Visual acuity and the severity of clinical presentation were assessed in both groups. Visual acuity, Snellen equivalent was 0.52 +/- 0.36 in group 1 and 0.82 +/- 0.39 in group 2 with lesions at the level of choroid. One-way analysis of variance ANOVA showed a statistically significant between-group difference in visual acuity (p = 0.03). The mean severity of clinical presentation was 11.80 +/- 2.04 points in group 1 and 5.80 +/- 4.18 points in group 2. T-test for independent samples yielded a statistically significant difference between the groups (p = 0.02). A statistically significant difference in visual acuity was the result of vasculitis in the group with the "candle-wax" phenomenon, which is associated with retinal vasculitis and causes cystoid macular edema and reduction of visual acuity. Complications such as cataract, glaucoma and neovascularization, which also decrease visual acuity, were more frequent in group 1. PMID:23115950
Jovanovi?, Svetlana V; Jovanovi?, Zorica D; Radoti?, Filip M; Sre?kovi?, Suncica B; Paunovi?, Svetlana S; Stojanovi?, Jasmina D
Avian simuliotoxicosis: outbreak in Louisiana.
From April 20 to May 10, 2010, multiple species of birds were seen at the Louisiana State University (LSU) Zoological Medicine Department for a disease syndrome characterized by acute lethargy, generalized subcutaneous petechiae, vasculitis, and death caused by a black fly identified as Simulium meridionale. Twenty psittacine birds presented with severe depression and multifocal subcutaneous hemorrhages over the body and especially noted in the featherless areas of the head and neck. Ten out of 20 clinical cases seen survived on a treatment regimen consisting of intravenous fluid therapy, anti-inflammatories, antihistaminic medications, and supportive care. The 10 other birds likely died of cardiopulmonary collapse and anaphylactoid reactions. In all post mortem examinations multifocal to coalescing dermal hemorrhage, eosinophilic dermatitis, severe edema, and vasculitis were observed. The LSU School of Animal Science conducted a brief survey that included 34 of the 64 Louisiana parishes and showed that 17 parishes, at least, were affected by this outbreak. A total of at least 225 poultry bird deaths could be attributed to black fly strikes. Simuliotoxicosis outbreaks have previously been reported in North America, and this report documents the clinical and post mortem findings, treatment response, and extent of such an outbreak in pet and farm birds in Louisiana. PMID:23050485
Schnellbacher, Rodney W; Holder, Kali; Morgan, Tim; Foil, Lane; Beaufr�re, Hugues; Nevarez, Javier; Tully, Thomas N
Cavia porcellus as a Model for Experimental Infection by Trypanosoma cruzi
The guinea pig (Cavia porcellus) is a natural reservoir for Trypanosoma cruzi but has seldom been used as an experimental infection model. We developed a guinea pig infection model for acute and chronic Chagas disease. Seventy-two guinea pigs were inoculated intradermally with 104 trypomastigotes of T. cruzi strain Y (experimental group); 18 guinea pigs were used as control group. Eight animals from the experimental group and two from the control group were sacrificed 5, 15, 20, 25, 40, 55, 115, 165, and 365 days after inoculation. During the acute phase (15 to 55 days), we observed parasitemia (with a peak on day 20) and positive IgM and IgG Western blots with anti-shed acute-phase antigen bands. The cardiac tissue showed vasculitis, necrosis (on days 40 to 55), moderate to severe inflammation, and abundant amastigote nests. Smaller numbers of amastigote nests were also present in kidney, brain, and other organs. In the early chronic phase (115 to 165 days), parasitemia disappeared and anti�T. cruzi IgG antibodies were still detectable. In cardiac tissue, the number of amastigote nests and the grade of inflammation decreased. In the chronic phase (365 days), the cardiac tissue showed vasculitis and fibrosis; detectable parasite DNA was associated with higher grades of inflammation. The experimental T. cruzi infection model in guinea pigs shows kinetics and pathologic changes similar to those of the human disease. PMID:21703410
Castro-Sesquen, Yagahira E.; Gilman, Robert H.; Yauri, Ver�nica; Angulo, Noelia; Verastegui, Manuela; Vel�squez, Daniel E.; Sterling, Charles R.; Martin, Diana; Bern, Caryn
A review on the vascular features of the hyperimmunoglobulin E syndrome.
Autosomal recessive, autosomal dominant and the sporadic forms of hyperimmunoglobulin E syndrome (HIES) are multi-system disorders. Although HIES patients may present with cold abscesses, the vascular features of HIES are not well recognized. The objective of this review is to characterize the nature and spectrum of vascular abnormalities in HIES patients. Vascular abnormalities in HIES patients were reviewed with Medline and Google Scholar-based searches. In brief, the searches combined terms related to HIES with the terms related to vasculature. Furthermore, reference lists from the original studies and review papers identified were screened. There were vascular abnormalities in 25 patients with HIES. These abnormalities were identified as aneurysms (coronary, aortic, carotid and cerebral), pseudoaneurysms, congenital patent ductus venosus, superior vena cava syndrome, vasculitides, vascular ectasia, thrombosis and others. They may be congenital or acquired, in the veins and arteries, affecting both sexes. These abnormalities can be seen in all subtypes of HIES. They could be also fatal in children and adults. Limited pathological investigations revealed the presence of vasculitis. Three of the patients were found to have overlap diseases. In this review, the spectrum of vascular abnormalities in HIES are documented and discussed in detail for the first time. They highlight a previously under-recognized and potentially devastating complication of these disorders. These vascular abnormalities constitute one of the major clinical characteristics in HIES. The presence of hypereosinophilia, vasculitis and defective angiogenesis in HIES may contribute to the formation of vascular abnormalities in HIES. PMID:19912258
Yavuz, H; Chee, R
Asymmetric dimethylarginine levels in patients with cutaneous anthrax: a laboratory analysis
Background Asymmetric dimethylarginine (ADMA), the main endogenous inhibitor of nitric oxide synthase, is considered to be associated with endothelial dysfunction. High ADMA levels have been shown to be related with disorders causing vascular inflammation such as hypertension, hypercholesterolemia, atherosclerosis, chronic heart failure, stroke and sepsis. Cutaneous anthrax (CA) is a serious infectious disease which may cause vasculitis. The aim of the study was to investigate the serum ADMA levels in patients with CA. Methods A total of 35 serum samples of the patients with CA and 18 control sera were tested for ADMA levels using ADMA ELISA kit (Immunodiagnostik AG, Bensheim, Germany). Results ADMA levels were found to be significantly higher in the patients group than the controls (p?0.001). In addition, ADMA levels were found to be positively associated with sedimentation rates (R?=?0.413; p?=?0.026), and inversely associated with international normalized ratio (INR) levels (R?=?-0.46; p?=?0.011). A cut-off value of 0.475 of ADMA had a sensitivity of 74.3%, specificity of 77.8%, and accuracy of 75.5% in the diagnosis of CA. Conclusion Although the exact mechanism still remains unclear, ADMA levels could be related to immune activation in CA. In addition, these data might suggest the higher ADMA levels in patients could be due to the perivascular inflammation and vasculitis in CA. PMID:24669818
FDG PET of infection and inflammation.
Nuclear medicine plays an important role in the evaluation of infection and inflammation. Fluorine 18 fluorodeoxyglucose (FDG) is a readily available radiotracer that offers rapid, exquisitely sensitive high-resolution tomography. In patients with acquired immunodeficiency syndrome, FDG positron emission tomography (PET) accurately helps localize foci of infection and is particularly useful for differentiating central nervous system lymphoma from toxoplasmosis. FDG PET can also help localize the source of fever of undetermined origin (FUO), thereby guiding additional testing. In the musculoskeletal system, FDG PET accurately helps diagnose spinal osteomyelitis, and in inflammatory conditions such as sarcoidosis and vasculitis, it appears to be useful for defining the extent of disease and monitoring response to treatment. FDG PET may be of limited usefulness in postoperative patients and in patients with a failed joint prosthesis or a tumor. Nevertheless, this relatively new imaging technique promises to be helpful in the diagnosis of infection and inflammation. FDG PET will likely assume increasing importance in assessing FUO, spinal osteomyelitis, vasculitis, and sarcoidosis and may even become the radionuclide imaging procedure of choice in the evaluation of some or all of these pathologic conditions. PMID:16160116
Love, Charito; Tomas, Maria B; Tronco, Gene G; Palestro, Christopher J
Gastrointestinal manifestations of Beh�et's disease.
Beh�et's disease is a rare vasculitis diagnosed by the presence of recurrent oral ulcers and two of the following: genital ulcers, typical eye lesions, typical skin lesions, and positive pathergy test. It is most commonly seen in countries along the ancient silk road from Eastern Asia to the Mediterranean Basin. Young adults between the second and fourth decades of life are mainly affected, with abdominal pain being the most common symptom. The ileocecal region is most commonly affected, with ulcerations that may penetrate or perforate. Rarely, the esophagus and stomach may have ulcerations. Bowel wall thickening is the most common finding on computed tomography (CT) scan. Pathology shows a vasculitis mainly involving the small veins or, alternatively, nonspecific inflammation. Corticosteroids, with or without other immunosuppressive drugs, are used for severe eye disease. Their use in intestinal disease is largely empirical. Surgery may be required for perforation. Beh�et's disease runs a chronic, unpredictable course with exacerbations and remissions which decrease in frequency and severity over time. Death is mainly due to major vessel disease and neurological involvement. PMID:18594975
Ebert, Ellen C
Branch retinal artery occlusion associated with posterior uveitis
Background The purpose of this study is to report the clinical features and visual outcome of branch retinal artery occlusion (BRAO) associated with posterior uveitis. This is a retrospective study including the 18 eyes of 18 patients. All patients underwent a complete ophthalmic evaluation. Fundus photography, fluorescein angiography, and visual field testing were performed in all cases. Results Diseases associated with BRAO included active ocular toxoplasmosis in 7 patients, rickettsiosis in 4, Beh�et�s uveitis in 2, West Nile virus infection in 1, idiopathic retinal vasculitis in 1, Crohn�s disease in 1, ocular tuberculosis in 1, and idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome in 1 patient. The mean initial visual acuity was 20/50. BRAO involved the first order retinal artery in 33.3% of the eyes, the second order retinal artery in 33.3%, an arteriole in 27.8%, and a cilioretinal artery in 5.5%. The macula was involved in 44.4% of the eyes and an acute focus of retinitis or retinochoroiditis was associated to BRAO in 55.5%. Repermeabilization of the occluded artery occurred in all patients with permanent scotomas in the corresponding visual field. The mean visual acuity at last visit was 20/32. Conclusions BRAO, with subsequent visual impairment, may occur in the eyes with posterior uveitis. Physicians should be aware of such vision-threatening complication of infectious and inflammatory eye diseases. PMID:23514435
Adamantiades-Behcet's disease-complicated gastroenteropathy.
Adamantiades-Behcet's disease (ABD) is a chronic, relapsing, systemic vasculitis of unknown etiology. It is more prevalent in populations along the ancient Silk Road from Eastern Asia to the Mediterranean Basin, and most frequently affects young adults between the second and fourth decades of life. ABD-complicated gastroenteropathy is a significant cause of morbidity and mortality, with abdominal pain as the most common symptom. The ileocecal region is affected predominantly, with ulcerations that may lead to penetration and/or perforation, whereas other parts of the gastrointestinal system including the esophagus and stomach can also be affected. Endoscopy is useful to locate the site and extent of the lesions, and tissue biopsy is often warranted to examine the histopathology that is often suggestive of underlying vasculitis of small veins/venules or, alternatively in some cases, nonspecific inflammation. Bowel wall thickening is the most common finding on computed tomography scan. Treatment is largely empirical since well-controlled studies are difficult to conduct due to the heterogeneity of the disease, and the unpredictable course with exacerbation and remission. Corticosteroids with or without other immunosuppressive drugs, such as cyclophosphamide, azathioprine, sulfasalazine, tumor necrosis factor ? antagonist or thalidomide should be applied before surgery, except in emergency. PMID:22363131
Wu, Qing-Jun; Zhang, Feng-Chun; Zhang, Xuan
Beh�et's disease is a multisystem inflammatory disorder that is most common in countries along the ancient "Silk Road". The eye is the most commonly involved vital organ in Beh�et's patients and the typical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Uveitis is the initial manifestation of the disease in 10-15% of the patients. Anterior uveitis is always nongranulomatous. Diffuse vitritis, retinal infiltrates, sheathing of predominantly retinal veins, and occlusive vasculitis are the typical signs of posterior segment inflammation. Spontaneous resolution of acute inflammatory signs is a diagnostic feature. Fundus fluorescein angiography is the gold standard in monitoring inflammatory activity. Laser flare photometry is a useful noninvasive tool since flare readings correlate with fluorescein angiographic leakage. The most common complications are cataract, maculopathy, and optic atrophy. Male patients have a more severe disease course and worse visual prognosis. Immunomodulatory therapy is indicated in all patients with posterior segment involvement. Corticosteroids combined with azathioprine and/or cyclosporine is used initially. Biologic agents, including interferon alfa and infliximab, are used in resistant cases. Visual prognosis has improved in recent years with an earlier and more aggressive use of immunomodulatory therapy and the use of biologic agents in resistant cases. PMID:20404988
Tugal-Tutkun, Ilknur
: The specific dermatoses of pregnancy represent a recently reclassified heterogeneous group of pruritic inflammatory skin diseases unique to pregnancy that include pemphigoid gestationis, polymorphic eruption of pregnancy (PEP), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy (AEP). Among them, PEP and AEP are the most frequent ones. We performed a histopathological study of a series of PEP and AEP patients (n = 41). Twenty-two patients had PEP that started in the third trimester in 16 (73%) patients and postpartum in 6 (27%) patients. Histopathology revealed a superficial or superficial and deep perivascular dermatitis with eosinophils in all biopsies and signs of a lymphocytic vasculitis in 5 (23%) cases. Epidermal changes, including epidermal hyperplasia, spongiosis, and parakeratosis, occurred in 8 cases, in particular in elder lesions. Nineteen patients had AEP that started earlier [less than third trimester, 14 (74%) patients; third trimester, 5 (26%) patients]. Clinically, 5 (26%) patients showed eczematous lesions, 7 (37%) papular lesions, 3 (16%) presented both eczematous and prurigo lesions, and 4 (21%) experienced exacerbation of preexisting atopic dermatitis. Histopathologically, AEP was characterized by a perivascular lymphohistiocytic infiltrate with frequent eosinophils (74%) and epidermal changes in all but most of P-type biopsies. No definitive differential histopathological criteria between PEP and AEP were found. Only lymphocytic vasculitis with a mixed infiltrate with eosinophils was more frequent in PEP patients. Timing of onset, morphology of skin lesions, and a detailed clinicopathologic correlation are essential for diagnosis. PMID:25243396
Massone, Cesare; Cerroni, Lorenzo; Heidrun, Neureiter; Brunasso, Alexandra M G; Nunzi, Enrico; Gulia, Andrea; Ambros-Rudolph, Christina M
Dermatopathology: LC07-1 PANNICULITIS.
The subcutaneous fat is a metabolic depot and also provides insulation from trauma. The subcutis is divided into lobules by fibrous septa. Within the fibrous septae course the small arteries, arterioles, veins and small nerve fibres. The nutrient artery supplies the centre of the lobule and drains to venules within the septum. Inflammatory disorders of the subcutaneous fat can be grouped into three main categories: 1) Septal panniculitis. 2) Lobular panniculitis. 3) Panniculitis associated with large vessel vasculitis.Most panniculitis are strictly speaking mixed but the classification is based on the predominant area of involvement.In septal panniculitis the inflammation is centred upon the connective tissue septa. There is often some spill over of the inflammatory cells into the periphery of the lobule. Erythema nodosum (EN) is the prototype of septal panniculitis. Other disorders associated with a septal panniculitis include necrobiosis lipoidica, factitial panniculitis, nephrogenic systemic fibrosis, cellulitis and microscopic polyangiitis.In the lobular panniculitides the inflammation is centered mainly within the lobule. Examples of lobular panniculitis include nodular vasculitis, lupus panniculitis, subcutaneous fat necrosis of the newborn, sclerema neonatorum, cold panniculitis, a1 anti-trypsin deficiency and pancreatic panniculitis. PMID:25188048
Calonje, Eduardo
Beh?et's Syndrome and Thrombosis
Beh�et syndrome (BS) is a multisystem vasculitis with unknown etiology and a unique geographic distribution. The disease course is characterized by exacerbations and remissions while abating as the years pass. The usual onset is in the third decade. Recurrent skin mucosa lesions and sight threatening panuveitis are the hallmark of the disease. Males are more severely affected than females. Vascular involvement can occur in up to 40% of cases. BS is unique among the vasculitides in that it may involve all sizes and types of vessels. It affects the veins more than the arteries. Lower extremity vein thrombosis is the most frequent manifestation of vascular involvement, followed by vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms. Vascular involvement is frequently associated with constitut onal symptoms and increased acute phase response and is the major cause of increased mortality. A predominantly neutrophilic vasculitis around the vaso vasorum is typical of BS. The thrombus is tightly adherent to the vessel wall which probably explains why thromboembolism is so rare despite the high frequency of venous disease. Thrombophilic factors do not seem to explain thrombotic tendency in BS. Immunosuppressive treatment is essential in suppression and preventing the attacks. PMID:21869912
Seyahi, Emire; Yurdakul, Sebahattin
In Vitro Global Gene Expression Analyses Support the Ethnopharmacological Use of Achyranthes aspera
Achyranthes aspera (family Amaranthaceae) is known for its anticancer properties. We have systematically validated the in vitro and in vivo anticancer properties of this plant. However, we do not know its mode of action. Global gene expression analyses may help decipher its mode of action. In the absence of identified active molecules, we believe this is the best approach to discover the mode of action of natural products with known medicinal properties. We exposed human pancreatic cancer cell line MiaPaCa-2 (CRL-1420) to 34??g/mL of LE for 24, 48, and 72 hours. Gene expression analyses were performed using whole human genome microarrays (Agilent Technologies, USA). In our analyses, 82 (54/28) genes passed the quality control parameter, set at FDR ? 0.01 and FC of ?�2. LE predominantly affected pathways of immune response, metabolism, development, gene expression regulation, cell adhesion, cystic fibrosis transmembrane conductance regulation (CFTR), and chemotaxis (MetaCore tool (Thomson Reuters, NY)). Disease biomarker enrichment analysis identified LE regulated genes involved in Vasculitis�inflammation of blood vessels. Arthritis and pancreatitis are two of many etiologies for vasculitis. The outcome of disease network analysis supports the medicinal use of A. aspera, viz, to stop bleeding, as a cure for pancreatic cancer, as an antiarthritic medication, and so forth. PMID:24454496
Subbarayan, Pochi R.; Sarkar, Malancha; Lokeshwar, Balakrishna L.; Ardalan, Bach
Objective To investigate if Lactobacillus casei cell wall extract (LCWE)-induced Kawasaki Disease (KD) accelerates atherosclerosis in hypercholesterolemic mice. Method and Resuslts Apoe?/? or Ldlr?/? mice were injected with LCWE (KD mice) or PBS, fed high fat diet for 8 weeks, and atherosclerotic lesions in aortic sinuses (AS), arch (AC) and whole aorta were assessed. KD mice had larger, more complex aortic lesions with abundant collagen, and both extracellular and intracellular lipid and foam cells, compared to lesions in control mice despite similar cholesterol levels. Both Apoe?/? KD and Ldlr?/? KD mice showed dramatic acceleration in atherosclerosis vs. controls, with increases in en face aortic atherosclerosis and plaque size in both the AS and AC plaques. Accelerated atherosclerosis was associated with increased circulating IL-12p40, IFN-?, TNF-?, and increased macrophage, DC, and T cell recruitment in lesions. Furthermore, daily injections of the IL-1Ra, which inhibits LCWE induced KD vasculitis, prevented the acceleration of atherosclerosis. Conclusions Our results suggest an important pathophysiologic link between coronary arteritis/vasculitis in the KD mouse model and subsequent atherosclerotic acceleration, supporting the concept that a similar relation may also be present in KD patients. These results also suggest that KD in childhood may predispose to accelerated and early atherosclerosis as adults. PMID:22628430
Chen, Shuang; Lee, Young Ho; Crother, Timothy R.; Fishbein, Michael; Zhang, Wenxuan; Yilmaz, Atilla; Shimada, Kenichi; Schulte, Danica J; Lehman, Thomas J.A.; Shah, Prediman K.; Arditi, Moshe
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that is generally restricted to medium-sized vessels. Here we describe the first case of a patient in which a bilateral testicular mass was a presenting symptom and the diagnosis was made on the basis of testicular histopathology. A 53-year-old Asian man presented with a history of constitutional symptoms and testicular swelling. Scrotal ultrasound revealed two avascular, bilateral, intratesticular lesions. The bilateral testicular abscess was treated without improvement. The patient developed left seventh cranial nerve palsy during his admission. The clinical changes made vasculitis or a related disorder more likely and the patient underwent a right testicular biopsy. Histopathology demonstrated features of transmural inflammation and fibrinoid necrosis of medium-sized vessel walls, consistent with PAN. This case illustrates the difficulty in diagnosing polyarteritis nodosa with isolated bilateral testicular swelling and the delay in the diagnosis. After 9 months of follow-up, no relapse had occurred and the patient's testosterone level was on the lower side of normal. PMID:21687399
Richards, Bethan; Rasiah, Kris; Thanigasalam, Ruban; Atmore, Bryn B; Laurent, Roger
Advances and challenges in the diagnosis and treatment of polymyalgia rheumatica
Polymyalgia rheumatica (PMR) is a common inflammatory condition that often affects people over the age of 50 years. Characteristic symptoms are shoulder and hip girdle pain and prolonged morning stiffness. Markers of inflammation are often elevated. Clinicians are often faced with the challenge of distinguishing PMR from other conditions, particularly rheumatoid arthritis and spondyloarthropathy that can mimic symptoms of PMR in older people. Additionally, there is an association between PMR and giant cell arteritis, a common large-vessel vasculitis which also affects people over the age of 50 years. Imaging of the large vessels in asymptomatic patients with PMR often reveals findings of subclinical vasculitis. Presently, there are no tests that are specific for the diagnosis of PMR and clinicians rely on a combination of history, physical examination, laboratory tests and imaging studies to make a diagnosis. A recent undertaking by the European League Against Rheumatism/American College of Rheumatology has led to the publication of provisional classification criteria of PMR. Ultrasonography, which is being increasingly used by rheumatologists, can greatly aid in the diagnosis of PMR and often shows changes of synovitis and tenosynovitis. Treatment consists of low doses of glucocorticoids which are associated with morbidity. Evaluation of newer biologic therapies targeting inflammatory cytokines is underway. Despite treatment, relapses are common. PMID:24489611
Warrington, Kenneth J.
NASA Astrophysics Data System (ADS)
Crohn's disease is an inflammatory bowel disease of unknown etiology. Vasculitis is hypothesized but it was never demonstrated in vivo. This study aimed to evaluate the vascular mucosa perfusion using fluorescence imaging in 13 patients who had previously undergone eileocolonic resection and who agreed to participate in a prospective endoscopic study of anastomotic recurrence. This anastomotic recurrence rate is known to be high (73% after 1 year follow-up) and is characterized by ulcerations. The fluorescence study was started with an I.V. bolus injection of sodium fluorescein. The pre-anastomotic mucosa was endoscopically examined with blue light that stimulates fluorescein fluorescence. Fluorescence emission was recorded with an ultra-high-sensitivity camera connected to the endoscope via an interference filter (520 - 560 nm). A uniform fluorescence was observed a few seconds after the injection and lasted for 15 min in healthy subjects. In case of recurrence, the centers of the ulcerations displayed a very low fluorescence indicating localized ischemia. In contrast, the rims of the ulcers revealed brighter fluorescent images than those of normal mucosa. The anastomotic ulcerations of Crohn's disease recurrence exhibit a high fluorescence intensity at their margins indicating an increased mucosal blood flow and/or enhanced transcapillary diffusion. These findings support the hypothesis of a primary vasculitis in Crohn's disease.
Mordon, Serge R.; Maunoury, Vincent; Klein, Olivier; Colombel, Jean-Frederic
Aim: To determine the incidence of immune complex associated complications (IAC) after severe meningococcal disease (SMD) in a group of Dutch children admitted to a paediatric intensive care unit (PICU). Methods: Retrospective chart analysis and follow up of 130 survivors of SMD admitted to PICU. Signs of IAC, inflammatory parameters, and temperature profile were reviewed. Results: Of 130 children with SMD, 20 (15.3%) showed one or more of the three manifestations of IAC: 18 (13.8%) developed arthritis (effusion, with or without erythema/arthralgia), 11 (8.4%) vasculitis, and five (3.8%) pleuritis. Eighteen of 20 (90%) patients with IAC had a secondary rise in temperature; in patients with no IAC this was 48 of 110 (43.6%). IAC was associated with leucocytosis in 82.3% versus 47.7% in patients without IAC, and with increased CRP in 86.6% versus 47.2% in patients without IAC. Leucocytes on admission were significantly lower in patients who would later develop IAC (mean 8.6 versus 13.8x109/l). Conclusion: IAC is a common complication of SMD, mainly occurring 4�10 days after systemic disease. IAC presents clinically as arthritis or vasculitis, mostly accompanied by secondary fever and raised inflammatory parameters. PMID:14500317
Goedvolk, C; von Rosenstiel, I A; Bos, A
Rheumatoid arthritis, spondyloarthropathies, and relapsing polychondritis.
The neurologic complications of rheumatic disease are highly variable and their manifestations are linked to the pathogenesis and clinical phenotype of the specific rheumatologic syndrome. In active rheumatoid arthritis (RA), the peripheral nervous system is most commonly involved and mononeuritis multiplex, nerve entrapment and vascultitic sensorimotor neuropathy are not uncommon. Central nervous system complications such as pachymeningitis and cerebral vasculitis are rare. TNF blockade therapy of RA is rarely associated with demyelinating syndromes. In the spondyloarthropathies, especially ankylosing spondylitis (AS), neurologic complications are more frequent in long-standing, advanced disease and include atlantoaxial subluxation, cauda equina syndrome, spinal stenosis, and acute vertebral fractures. Peripheral nervous system involvement in any of the spondyloarthropaties is rare. Relapsing polychondritis (RP) is characterized by recurring bouts of inflammation, destruction of cartilaginous structures, and systemic and rarely central nervous system vasculitis. Visual-oculo and auditory complications are common. Definitive treatment of the neurologic complications and prevention of subsequent ones is dependent upon effective treatment of RA, AS or RP. PMID:24365312
Ostrowski, Rochella A; Takagishi, Troy; Robinson, John
IgG4-related disease (IgG4-RD) is a systemic inflammatory disease characterized by marked infiltration of IgG4-positive (+) plasma cells into affected organs, but the concept of this disease has only recently been established. Coronary vasculitis is a rare disease that can cause sudden death, and it has recently been reported that IgG4-RD may be associated with vasculitis, including periarteritis and coronary disease. In this paper we report an autopsy case of sudden death of a man in his thirties, in which coronary periarteritis with features of IgG4-related periarteritis was detected. IgG4-RD was suspected from the presence of the following histopathological features: (1) markedly thickened adventitia and marked infiltration of the adventitia and periarterial fat by lymphocytes and plasma cells; and (2) infiltration of IgG4-positive plasma cells (ratio of IgG4+ cells to IgG4+ cells of >40 %, 50 IgG4+ plasma cells per high-power field) on immunostaining. The etiology and pathophysiology of IgG4-RD and IgG4-related periarteritis are still unclear, and further investigation of these conditions and their association with coronary lesions is needed. Careful consideration should be given to the possible presence of IgG4-RD when forensic pathologists encounter cases of sudden death accompanied by coronary periarteritis. PMID:24337948
Inokuchi, Go; Hayakawa, Mutsumi; Kishimoto, Takashi; Makino, Yohsuke; Iwase, Hirotaro
Proapoptotic Bak and Bax guard against fatal systemic and organ-specific autoimmune disease
Dysregulation of the �intrinsic� apoptotic pathway is associated with the development of cancer and autoimmune disease. Bak and Bax are two proapoptotic members of the Bcl-2 protein family with overlapping, essential roles in the intrinsic apoptotic pathway. Their activity is critical for the control of cell survival during lymphocyte development and homeostasis, best demonstrated by defects in thymic T-cell differentiation and peripheral lymphoid homeostasis caused by their combined loss. Because most bak?/?bax?/? mice die perinatally, the roles of Bax and Bak in immunological tolerance and prevention of autoimmune disease remain unclear. We show that mice reconstituted with a Bak/Bax doubly deficient hematopoietic compartment develop a fatal systemic lupus erythematosus-like autoimmune disease characterized by hypergammaglobulinemia, autoantibodies, lymphadenopathy, glomerulonephritis, and vasculitis. Importantly, these mice also develop a multiorgan autoimmune disease with autoantibodies against most solid glandular structures and evidence of glandular atrophy and necrotizing vasculitis. Interestingly, similar albeit less severe pathology was observed in mice containing a hematopoietic compartment deficient for only Bak, a phenotype reminiscent of the disease seen in patients with point mutations in BAK. These studies demonstrate a critical role for Bak and an ancillary role for Bax in safeguarding immunological tolerance and prevention of autoimmune disease. This suggests that direct activators of the intrinsic apoptotic pathway, such as BH3 mimetics, may be useful for treatment of diverse autoimmune diseases. PMID:23349374
Mason, Kylie D.; Lin, Ann; Robb, Lorraine; Josefsson, Emma C.; Henley, Katya J.; Gray, Daniel H. D.; Kile, Benjamin T.; Roberts, Andrew W.; Strasser, Andreas; Huang, David C. S.; Waring, Paul; O'Reilly, Lorraine A.
Feline infectious peritonitis in a mountain lion (Puma concolor), California, USA.
Feline infectious peritonitis (FIP) is a fatal immune-mediated vasculitis of felids caused by a mutant form of a common feline enteric virus, feline enteric coronavirus. The virus can attack many organ systems and causes a broad range of signs, commonly including weight loss and fever. Regardless of presentation, FIP is ultimately fatal and often presents a diagnostic challenge. In May 2010, a malnourished young adult male mountain lion (Puma concolor) from Kern County, California, USA was euthanized because of concern for public safety, and a postmortem examination was performed. Gross necropsy and histopathologic examination revealed necrotizing, multifocal myocarditis; necrotizing, neutrophilic, and histiocytic myositis and vasculitis of the tunica muscularis layer of the small and large intestines; and embolic, multifocal, interstitial pneumonia. Feline coronavirus antigen was detected in both the heart and intestinal tissue by immunohistochemistry. A PCR for coronavirus performed on kidney tissue was positive, confirming a diagnosis of FIP. Although coronavirus infection has been documented in mountain lions by serology, this is the first confirmed report of FIP. PMID:23568918
Stephenson, Nicole; Swift, Pamela; Moeller, Robert B; Worth, S Joy; Foley, Janet
Protracted superficial Wegener granulomatosis in a child.
Wegener granulomatosis is a rare, chronic, multisystemic vasculitis affecting mainly the upper and lower respiratory tracts together with glomerulonephritis, but the disease may involve any other organ. Protracted superficial form is a rare variant of the disease. We report a case of protracted superficial Wegener granulomatosis in a 16-year-old boy in whom the disease started with recurrent digital ulcers at age 7 years. Later, he developed nodules and papules associated with upper airway involvement and ocular keratitis without lung or renal involvement. C-ANCA was positive. The patient was treated with oral prednisone. Similar cutaneous and mucosal lesions developed during two relapses of the disease without renal or respiratory involvement. PMID:18319011
Ben Ghorbel, Imed; Dhrif, Asma Sioud; Miled, Mohamed; Houman, Mohamed Habib
Current options for the treatment of optic neuritis
Optic neuritis can be defined as typical (associated with multiple sclerosis, improving independent of steroid treatment), or atypical (not associated with multiple sclerosis, steroid-dependent improvement). Causes of atypical optic neuritis include connective tissue diseases (eg, lupus), vasculitis, sarcoidosis, or neuromyelitis optica. In this manuscript, updated treatment options for both typical and atypical optic neuritis are reviewed. Conventional treatments, such as corticosteroids, therapeutic plasma exchange, and intravenous immunoglobulin therapy are all discussed with commentary regarding evidence-based outcomes. Less commonly used treatments and novel purported therapies for optic neuritis are also reviewed. Special scenarios in the treatment of optic neuritis � pediatric optic neuritis, acute demyelinating encephalomyelitis, and optic neuritis occurring during pregnancy � are specifically examined. PMID:22927730
Pula, John H; MacDonald, Christopher J
Pediatric Inflammatory Diseases. Part IV: Miscellaneous, Reye, PRES, Sarcoidosis.
Some rare neurological diseases affecting children have no well defined etiology and pathogenetic mechanisms. In this article diseases like Reye syndrome, Beh�et disease, pediatric neurosarcoidosis, Posterior Reversible Encephalopathy Syndrome are described. Some of the main neuroradiological differential aspects are also critically considered. Reye syndrome is characterized by symmetric thalamic, white matter and basal ganglia lesions, in children with recent history of salycilates or immunosuppressive drugs intake. The most typical MRI feature of neurosarcoidosis is basilar meningeal thickening and enhancement with intraparenchymal enhancing nodules and white matter focal abnormalities. The classical distribution of lesions helps differential diagnosis with infectious meningoencephalitis. Differential diagnosis with relapsing-remitting multiple sclerosis his helped by the evidence of meningeal abnormalities. Neuro-Beh�et is characterized by mesodiencephalic lesions in children with encephalopathy and coexistence of oral and genital ulcers and ocular abnormalities. PRES can be differentiated from vasculitis for the typical posterior white matter involvement and the different clinical features. PMID:24029186
Gallucci, M; Smith, J D; Limbucci, N; Rossi, A; Demaerel, P; Krings, T; D'Amico, A; Micheli, C
Portal vein and aortic thromboses in a Siberian husky with ehrlichiosis and hypothyroidism.
A six-year-old, neutered male Siberian husky was presented for euthanasia for end-stage liver disease. Examination of the dog raised questions regarding the severity of the condition. It had presented to the referring veterinarian with polyuria, polydipsia and weight loss. Blood tests at that time revealed elevated liver enzymes and hypoalbuminaemia. Cirrhosis was presumptively diagnosed, based on an ultrasound examination, which showed ascites with a normal liver. The dog had a history of hypothyroidism, which was controlled with levothyroxine. Physical examination revealed cachexia. A second abdominal ultrasound examination was performed and revealed portal vein and aortic thromboses. Tick titres showed a positive Ehrlichia canis titre of 1:640. Skin biopsies showed lymphoplasmacytic vasculitis. Doxycycline and aspirin treatment was initiated, and the clinical signs resolved. The authors concluded that the thrombi were primarily caused by chronic ehrlichiosis, based on the clinical findings and the response to treatment. PMID:14510331
Bressler, C; Himes, L C; Moreau, R E
Multi-system mitochondrial disorder with recurrent steroid-responsive eosinophilia.
Though mitochondrial disorders due to a respiratory chain defect may manifest with haematological abnormalities such as anaemia, neutropenia, or thrombocytopenia, recurrent steroid-responsive eosinophilia has not been reported as a manifestation of a mitochondrial disorder. In a polymorbid 65-year old female recurrent erythema, asthma, chronic bronchitis, gastritis with eosinophilic granuloma, recurrent episodes of hypereosinophilia, and polyarthralgia suggested Churg�Strauss syndrome, which was excluded upon absence of eosinophilic vasculitis on any of the biopsies. However, corticosteroids were effective for hypereosinophilia. In addition to the hypereosinophilic syndrome a mitochondrial disorder with multi-system affection was diagnosed upon the clinical presentation and a muscle biopsy indicative of a mitochondrial disorder. Hypereosinophilia was interpreted as a manifestation of the mitochondrial disorder after exclusion of various differentials. Mitochondrial disease may go along with marked eosinophilia mimicking Churg�Strauss syndrome. Steroids may be useful to resolve episodic eosinophilia but may be ineffective for other features of mitochondrial disorders. PMID:19370352
Finsterer, Josef; H�ger, Franz
Ocular toxoplasmosis: a very rare presentation in an immunocompetent patient.
A 28-year-old man presented with a 2-week history of red eye, photophobia, pain and decreased visual acuity of the right eye. The ophthalmological examination revealed hypertensive non-granulomatous panuveitis, retinal vasculitis with focus of retinochoroiditis with pigmented central area suggestive of ocular toxoplasmosis in the active phase. He started treatment with azithromycin, pyrimethamine, topical steroids and measures for control of intraocular pressure. Serology for Toxoplasma gondii was positive and for HIV, negative. For headache and vomiting, he was hospitalised in order to exclude cerebral toxoplasma. The cerebral CT scan, MRI and lumbar puncture were negative and treatment was changed to pyrimethamine, sulfadiazine and prednisolone. For persistence of vomiting he started clindamycin with clinical and ophthalmological improvement. The patient is currently under prophylaxis with co-trimoxazol for 1?year, and maintains clinical improvement. This case illustrates the rarity of presentation of ocular toxoplasmosis, without cerebral in an immunocompetent patient. PMID:25270156
Matias, Margarida; Gomes, Antonia; Marques, Tiago; Fonseca, Ana Claudia
A Case of Cerebellar Ataxia Associated with HIV Infection.
Cerebellar complications of HIV infection primarily manifested in ataxia, usually arise as the result of cerebellar lesions due to opportunistic infections, vasculitis or neoplastic processes. A 28 year old female known to have HIV infection for last four years, presented to our hospital with progressive unsteadiness in walking, slurring of speech and intention tremors for the last two months. There was no family history of similar complaints, and she was on Anti retroviral treatment for last one and a half years. The results of examination were notable for severe dysarthria, slow saccades, a conspicuous dysmetria and dysdiadokokinesia. She had no cognitive, sensory or motor deficits. MRI revealed diffuse cerebellar atrophy. Extensive laboratory work up failed to disclose a cause for subacute ataxia. Isolated cerebellar degeneration in an HIV patient is rare and should prompt a diagnostic work up. PMID:24759449
Anand, Kuljeet Singh; Wadhwa, Ankur; Garg, Jyoti
[Systemic vasculitides: revised nomenclature, new therapeutic strategies].
Vasculitides comprise a heterogeneous group of inflammatory diseases of the blood vessels, which are characterized both by their diversity and an overlap of clinical and pathological findings and manifestations. The etiology of most vasculitides is not yet clear. Their nomenclature and classification is therefore a challenge for internists, immunologists, and pathologists. The revised Chapel Hill Consensus Conference (CHCC) 2012 nomenclature includes an update of the names and definitions of the CHCC 1994 nomenclature. In addition, four new categories were introduced to describe the spectrum of vasculitis comprehensively. Because of the side effects of cytotoxic immunosuppressive agents there is a need for safer and more effective targeted therapies. The first results from studies using biologicals in the treatment of vasculitides are promising. PMID:23512366
Lamprecht, P
Thoracoscopic cervicodorsal sympathectomy with diathermy.
This study reports our experience of using thoracoscopic cervicodorsal sympathectomy with diathermy. From December 1994 to September 1998, we performed 53 thoracoscopic sympathectomies in 35 patients. There were 15 men and 20 women, ages 18 to 61 years. Ten surgeries were performed on the right side, 7 were the left, and 18 were bilateral. Indications for surgery were causalgia/reflex sympathetic dystrophy in 8 patients, Raynaud's/vasculitis in 6, intractable Raynaud's disease in 4, and hyperhydrosis in 17 (bilateral procedure). Operating time ranged from 10 to 50 min for unilateral procedures and from 45 to 80 min for bilateral procedures. Patients stayed in the hospital 1 to 4 days. From favorable immediate and follow-up results we conclude that thoracoscopic cervicodorsal sympathectomy using diathermy is feasible, safe, and effective. PMID:10541610
Cartier, B; Cartier, P
Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic small vessel vasculitis associated with asthma and eosinophilia. Optimal therapy for maintenance of remission is yet to be defined. We present a case-series of three patients with EGPA in whom IFN-?, an immunomodulatory cytokine induced remission, which was maintained even after discontinuation of the drug. In all patients (ages 60, 51, and 50 years), remission was associated with normalisation of eosinophil counts and IgE-levels. Moreover, the patients remained in remission for one to four years. Two patients did not need further immunosuppression, one patient required low dose maintenance therapy. Although reversible side effects occur, IFN-?-therapy induces long-term remission of EGPA even after discontinuation of treatment.
Seeliger, B.; Foerster, M.; Neumann, T.; Moeser, A.; Happe, J.; Kehler, N.; Kroegel, C.
Immunosuppressive and cytotoxic therapy: pharmacology, toxicities, and monitoring.
Treatment strategies for anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are evolving. Cyclophosphamide (CYC) plus corticosteroids (CSs) is the mainstay of therapy for generalized, multisystemic AAV. Historically, the combination of CYC plus CS was used for a minimum of 12 months, but concern about late toxicities associated with CYC has led to novel treatment approaches. Currently, short-course (3 to 6 months) induction treatment with CYC plus CS, followed by maintenance therapy with less toxic agents (eg, methotrexate, azathioprine, mycophenolate mofetil) is recommended. Further, methotrexate combined with CS may be adequate for limited, non-life-threatening AAV. Recent studies suggest that rituximab may be useful for induction therapy or for CYC-refractory AAV. This article reviews the key agents used to treat AAV, with a focus on pharmacology, toxicities, and monitoring. PMID:21674420
Wang, Tisha; Weigt, S Sam; Belperio, John A; Lynch, Joseph P
Cryptogenic embolic stroke in a girl with a trisomy 21 mosaic.
Stroke in trisomy 21 may be due to cardioembolism, atherosclerosis, vasculitis, moyamoya disease, sinus venous thrombosis, internal carotid hypoplasia or infections like endocarditis with septic emboli, meningitis or brain abscess. In rare cases, however, stroke etiology remains unexplained. We present a 19 year old Caucasian girl with trisomy 21 with a 47XX+21 karyotype who suffered at age 11�years from a transient ischemic attack with left hemiparesis, and at age 17�years from an ischemic stroke in the territory of the right cerebral medial artery. She suffered from arterial hypertension, obesity and hypercholesterolemia. Since blood coagulation studies, immunologic parameters, blood cultures, 24-h Holter monitoring, transthoracic and transesophageal echocardiography, magnetic resonance angiography of the extra- and intracranial vessels, thoracic and abdominal aorta and renal arteries did not provide any explanation for the stroke, implantation of a loop recorder is considered in order to detect episodes of clinically silent atrial fibrillation. PMID:22426657
St�llberger, Claudia; Weiss, Simone; Zlabinger, Gerhard; Finsterer, Josef
Neuro-beh�et disease and autoinflammatory disorders.
Misregulation of innate immunity leads to autoinflammation. Beh�et disease is an autoinflammatory condition involving recurrent attacks of inflammation in skin, eyes, joints, and even the nervous system. The etiology may involve vascular inflammation. Central nervous system involvement in neuro-Beh�et disease (NBD) comes in the form of parenchymal NBD or nonparenchymal NBD. The parenchymal form has a predilection for the brainstem, diencephalon and cerebral hemispheres, and represents a meningoencephalitis thought to be related to small vessel vasculitis. Cerebral venous sinus thrombosis, arising from a vasculitic process of large veins, comprises the majority of vascular NBD cases. The rarer monogenetic autoinflammatory syndromes are characterized by periodic fever, and typically present in the pediatric population. Neurologic involvement in these syndromes typically presents in the form of an aseptic meningitis. Treatment of autoinflammatory disorders involves immune modulation with corticosteroids, disease-modifying antirheumatic medications, and increasingly antibodies targeting cytokines like tumor necrosis factor ? and interleukin 1. PMID:25369439
Miller, Julie J; Venna, Nagagopal; Siva, Aksel
Granuloma faciale: a rare disease from a dermoscopy perspective*
The granuloma faciale is a rare and benign skin disease of unknown etiology, characterized by chronic leukocitoclastic vasculitis. It is characterized by skin lesions predominantly facial whose course is chronic and slowly progressive. The diagnosis is based on clinical features, histopathology and, more recently, in dermoscopy. We describe the case of a male patient, 40 years old, with a sarcoid lesion on the malar site, whose histopathological examination revealed a mixed inflammatory infiltrate with presence of Grenz zone. Dermoscopy revealed a pink background with white striations. The definitive diagnosis is made by histopathologic evaluation, and dermatoscopy can be helpful. It is known to be resistant to therapy, oral medications, intralesional and surgical procedures are options. PMID:24346891
Teixeira, Danilo Augusto; Estrozi, Bruna; Ianhez, Mayra
Wegener's Granulomatosis: A Model of Auto-antibodies in Mucosal Autoimmunity
Wegener's granulomatosis (WG) is an autoimmune condition marked by vasculitis of small and medium sized vessels particularly affecting the upper respiratory tract and kidneys. There is a strong mucosal component similar to other autoimmune conditions such as systemic lupus erythematosus and Beh�et's disease. While the pathogenesis of WG is not completely known, auto-antibodies such as IgG ANCAs have been implicated in endovascular damage and modulation of neutrophil / monocyte responses by Fc receptor (FcR) signaling. Due to the substantial mucosal involvement in WG (oral, nasal, and upper respiratory tract involvement), it is probable that IgA antibodies (perhaps IgA ANCAs) play a role in disease. Given discrepancies in associating ANCA levels with disease activity, future work should determine if IgA ANCAs are present in WG patients and examine the biology underlying the ANCAs' signaling partners - the FcRs. PMID:19482554
Kelley, James M.; Edberg, Jeffrey C.; Kimberly, Robert P.
Moyamoya syndrome in a known case of pulmonary tuberculosis
We report an unusual association of pulmonary tuberculosis with moyamoya syndrome in a 30-year-old Filipino female who was admitted to our hospital with a 1-week history of fever and cough. Chest X-ray showed widespread bilateral consolidation with cavity, whereas sputum was positive for acid fast bacilli (AFB). Two weeks after starting antituberculous treatment, the patient developed two episodes of loss of consciousness, which were unwitnessed. Urgent brain computed tomography (CT) showed multiple infarctions, suggesting vasculitis. The electroencephalogram showed epileptic discharges. Magnetic resonance angiography showed a picture consistent with moyamoya disease. Brain CT angiography was performed and it showed the same pictures. The patient was diagnosed with pulmonary tuberculosis-associated moyamoya syndrome. On the following days, she was discharged on antituberculous medications, antiepileptic and oral hypoglycemic treatment. After 1 year, the patient was seen in the clinic, she was well and seizure-free. PMID:21808515
Khan, Fahmi Yousef; Kamal, Hussain; Musa, Rania; Hayati, Ahmed
Neutrophilic dermatoses: an update.
Neutrophilic dermatoses constitute a heterogeneous group of dermatologic diseases, which are unified by the predominance of neutrophils within the inflammatory infiltrate on histopathology. The aims of this review were to provide an update on the clinical and histologic presentation of the main neutrophilic dermatoses and to develop a guide for clinical practice. A structured literature search of PubMed, Medline, and Embase was performed, using the key words "neutrophilic disorders", "cutaneous small vessel vasculitis", "Sweet's syndrome", "bowel associated dermatosis arthritis syndrome", "Behcet's", "palisaded neutrophilic and granulomatous dermatosis", "rheumatoid neutrophilic dermatitis", and "pyoderma gangrenosum". Related articles were screened for key terms and were included if appropriate. This group contains a wide spectrum of unique disorders, each with its own histologic and clinical subtleties, making specific diagnosis of a given entity within the group diagnostically challenging. The fact that overlapping forms of neutrophilic dermatoses, which share features of multiple neutrophilic dermatoses, are not uncommon makes the diagnoses more challenging. PMID:25154386
Alavi, Afsaneh; Sajic, Dusan; Cerci, Felipe B; Ghazarian, Danny; Rosenbach, Misha; Jorizzo, Joseph
Common dermatologic manifestations of primary immune deficiencies.
The skin is the largest organ of our body; it consists of the epidermis, dermis, hair follicles, sweat glands, blood vessels, and connective tissue matrix. Its main function is to act as a barrier to the outside world and protect us from infections. Any component of the skin is subject to insults from the environment and/or from within the body. Primary immune deficiency patients present with recurrent or prolonged infections not frequently seen in healthy individuals. Oftentimes, these infections involve the skin. Primary immune deficiency may also present with noninfectious cutaneous signs, such as eczema; erythroderma; granulomas; dysplasia of the skin, hair, nails, or teeth; pigmentary changes; angioedema; urticaria; vasculitis; or autoimmune skin disease due to immune dysregulation. Prompt recognition of the underlying diagnosis and initiation of treatment decrease morbidity. This review provides the reader with an up-to-date summary of the common dermatologic manifestations of primary immune deficiency diseases. PMID:25269404
Relan, Manisha; Lehman, Heather K
Cerebral tumor or pseudotumor?
Pseudotumoral lesions are uncommon but important to identity lesions. They can occur during inflammatory diseases (systemic diseases, vasculitis, demyelinating diseases), infectious, and vascular diseases. Also, in a patient with a treated tumor, pseudo-progression and radionecrosis must be differentiated from the tumoral development. Diagnosis can be difficult on an MRI scan, but some MRI aspects in conventional sequences, diffusion, perfusion and spectroscopy can suggest the pseudotumoral origin of a lesion. Imaging must be interpreted according to the context, the clinic and the biology. The presence of associated intracranial lesions can orientate towards a systemic or infectious disease. A T2 hyposignal lesion suggests granulomatosis or histiocytosis, especially if a meningeal or hypothalamic-pituitary involvement is associated. Non-tumoral lesions are generally not hyperperfused. In the absence of a definitive diagnosis, the evolution of these lesions, whether under treatment or spontaneous, is fundamental. PMID:25260711
Leclercq, D; Trunet, S; Bertrand, A; Galanaud, D; Leh�ricy, S; Dormont, D; Drier, A
Adult onset Still's disease: clinical experience with 18 patients over 15 years in northern India.
Over a 15 year period 18 patients (eight men, 10 women), 16-50 years old, were diagnosed as having adult onset Still's disease. Fever and arthralgia were always present but prominent lymphadenopathy was uncommon and the serosa were rarely affected. The typical rash of this disease was observed in nine patients. Several complications, including deforming arthritis, amyloidosis, granulomatous hepatitis, uveitis, scleritis, cutaneous vasculitis, and cardiomyopathy, were observed during follow up. Two patients were affected by a nosocomial infection during immunosuppressive treatment for uncontrolled disease. There were no characteristic features at necropsy. Ten patients had a monocyclic course that responded well to aspirin and indomethacin, whereas eight had a polycyclic pattern which invariably required treatment with corticosteroids. Serious complications developed exclusively in the latter group. This group of patients requires early, intensive disease modifying treatment. PMID:1586255
Bambery, P; Thomas, R J; Malhotra, H S; Kaur, U; Bhusnurmath, S R; Deodhar, S D
Beh�et's syndrome--classification criteria.
Beh�et's syndrome, classified as a vasculitis, is a multisystem disease of uncertain pathogenesis without any specific diagnostic feature or laboratory investigation. Diagnosis, therefore, depends on the grouping together of sufficient clinical manifestations for the clinician to be convinced of the diagnosis. Over many years various sets of diagnostic criteria have been proposed. In 1989 international criteria were proposed, which are better known as classification criteria, which depend on the presence of recurrent oral ulceration plus two of recurrent genital ulceration, typical eye lesions, typical skin lesions or a positive pathergy (skin hyperreactivity) test. It is emphasized that these are criteria for the classification of groups of patients participating in research programmes to ensure comparability of the groups, and not for the diagnosis of the individual patient in the clinical situation. PMID:10615533
Barnes, C G
Case of scrub typhus complicated by severe disseminated intravascular coagulation and death.
Scrub typhus is an infectious disease that is caused by Orientia tsutsugamushi. The authors describe an autopsied case of scrub typhus complicated with severe disseminated intravascular coagulation (DIC). An 82-year-old man complained of fever 4?days after climbing a mountain. The patient was admitted to an urban hospital, and meropenem and ceftriaxone were administered. The patient's condition deteriorated and he was transferred to a second hospital. On physical examination, a black scab was found and scrub typhus was suspected. Despite intensive treatment, the patient died on the fifth day. High levels of O.?tsutsugamushi IgM antibody were confirmed. An autopsy revealed systemic vasculitis and perivasculitis. The endothelial tissue of the white pulp of the spleen was markedly infiltrated by plasma cells. The authors speculated that a severe immune reaction against O.?tsutsugamushi enhanced an inflammatory response, leading to DIC. This case is a warning to doctors who are not familiar with scrub typhus. PMID:23039302