takayasu arteritis ( ta ) is a rare chronic inflammatory disease with a granulomatous panarteritis consisting of thickening , inflammatory infiltration , and hyperplasia of the arterial wall1 ) . posterior reversible encephalopathy syndrome ( pres ) is a rare neuroradiologic condition associated with headache , seizure , visual disturbances , and focal neurological deficit2 ) . most cases of pres have been described in conditions with hypertension , immunosuppressive drug use , and autoimmune diseases2 ) . complete recovery is usually possible if the syndrome is appropriately diagnosed and the associated condition is well controlled2 ) . the pathophysiology of pres involves endothelial dysfunction , altered cerebral vasoregulation , and vasospasm with subsequent ischemia2 ) . ta is a vasculitis with hypertension and hence can lead to the development of pres . we reported a case of a 5-year - old girl who presented with pres caused by presumed ta . the duration of seizure was approximately 10 minutes and was controlled with anticonvulsant injections in another hospital . she was previously healthy and had no history of epilepsy , hypertension , or renal disease . she suffered from a sudden onset of vomiting , headache and abdominal pain 1 week ago . she had no chest or back pain , and no history of limb claudication . on admission , the subject 's blood pressure was 170/103 mmhg , pulse rate was 94 beats per minute , and respiratory rate was 24 breaths per minute . height and body weight was 119.2 cm ( 95th percentile ) and 22.3 kg ( 95th percentile ) , respectively . auscultation revealed no gallop rhythm , abnormal heart sounds , or bruits over the abdomen . laboratory findings were as follows : hemoglobin , 14.8 g / dl ; erythrocyte sedimentation rate ( esr ) , 17 mm / hr ; c - reactive protein ( crp ) , 0.27 mg / dl ; blood urea nitrogen / creatinine , 9.0/0.73 mg / dl ; total protein / albumin , 8.4/5.2 g / dl ; serum cholesterol , 188 mg / dl ; igg / iga / igm 929/123/115 mg / dl ; serum sodium , 138 meq / l ; potassium , 4.0 meq / l ; and chloride , 95 meq / l . serum c3 and c4 levels were normal . serum autoantibodies including antinuclear antibody , anti - dsdna , antineutrophilic cytoplasmic antibody , and lupus anticoagulant were negative . the levels of serum aldosterone and renin activity were 85.1 ng / dl ( normal range , 127.3 ng / dl ) and 59.9 ng the findings of brain magnetic resonance imaging ( mri ) were compatible with pres ( fig . the findings of electroencephalography were suggestive of diffuse cerebral dysfunction . sustained hypertension with elevated plasma renin activity and normal renal function in the patient , suggested the possibility of renovascular hypertension . computed tomography ( ct ) was performed to find out the cause of renovascular hypertension . 2 ) . dimercaptosuccinic acid ( dmsa ) renal scan showed no isotope uptake in the left kidney . after the admission , blood pressure was not controlled , and intravenous antihypertensive medication was injected and the dosage was gradually increased to control hypertension ( nicardipine 1 mcg / kg / min and labetalol 3 mg / kg / hr ) . the patient showed the normal blood pressure ( 100/54 mmhg ) , the alert mentality and no seizure with intravenous antihypertensive medication . the pediatric nephrology and urology team made a collaborative decision to do perform a nephrectomy for evaluation of the mass lesion pathology and to control the hypertension . thirteen days after her admission , left nephrectomy with laparoendoscopic single site surgery was performed . at 1-week follow - up after operation , the levels of serum aldosterone and renin activity were 1.9 ng / dl and 0.48 ng / ml , respectively . the renal artery showed focal endovascular granulation tissue and perivascular fibromuscular proliferation on pathologic evaluation ( fig . these findings are not pathognomic , but suggestive of localized involvement of vasculitis , such as ta . her blood pressure improved dramatically postoperatively , and she was discharged with angiotensin - converting - enzyme inhibitor ( enalapril 0.1 mg / kg / day ) because she intermittently showed the blood pressure above 113/74 mmhg , which is the 95th percentile level of blood pressure by age and height percentile . her blood pressure and renal function remained normal over 20 months without antihypertensive or immunosuppressant medication . ta is a systemic vasculitis of large vessels that mainly involves the aorta and its branches , usually presenting in the third decade of life3 ) . although the common presenting symptom of ta is hypertension , rare cases have reported hypertensive encephalopathy and pres as presenting symptoms . the pathophysiology of pres is explained by 2 conditions ; hypertension leading to increased capillary filtration , or endothelial dysfunction resulting in an altered blood - brain barrier and the development of cerebral edema2 ) . our patient 's inflammatory state and severe hypertension due to renal artery occlusion were presumably sufficient to develop pres . recent data from camara - lemarroy et al.4 ) showed that there are 10 previous cases of ta presenting with pres in the literature , and the age of onset ranged from 9 to 29 years . our case is the youngest age of the reported cases of ta presenting with pres . diagnosis of ta during childhood remains challenging and may be delayed because of its rare incidence and the nonspecific symptoms3 ) . an earlier case report by mahmud et al.5 ) described a 5-year - old girl with ta who presented with a headache , generalized edema , hypertension , proteinuria , and minimally functioning kidneys . aypar et al.6 ) reported about a 4-year - old girl with ta - induced hypertension treated with oral corticosteroids . although these cases showed typical findings of ta according to angiography , our case revealed no evidence of other large vessel involvement beside the renal artery . the takayasu conference proposed a classification based on angiographic abnormalities , and similar cases of ta with only renal artery involvement are classified as ta type ii8 ) . furthermore , there are some differences in vascular involvement and the incidence of aneurysms among different populations9 ) . a comparative study has shown that japanese patients more frequently have lesions at the aortic arch and/or its branches ( 99% of 96 cases ) , while the most common lesion in korean ( 76% of 109 cases ) and indian patients ( 92% of 50 cases ) is the abdominal aorta . additionally , renal artery involvement is a potential manifestation of ta that has been found to occur in 60% of the patients in india and korea9 ) . the european league against rheumatism / paediatric rheumatology european society ( eular / pres ) proposed that ta consists of an angiographic abnormality and at least one of the following four features : decreased pulses / claudication , blood pressure difference of > 10 mmhg , bruit , and hypertension3 ) . while the angiographic abnormality in this case was not a typical finding of ta , ta was a possible diagnosis according to the eular / pres proposal , as the renal artery was affected and due to the pathologic findings in our case . because renovascular hypertension is usually be caused by fibromuscular dysplasia ( fmd ) fmd is defined as a nonatherosclerotic , noninflammatory vascular disease and medial fibroplasia is a common finding3 ) . on the other hand , ta is a chronic granulomatous inflammation and the vascular pathology in ta is characterized by segmental and patchy granulomatous inflammation of all three layers of the large vessels3 ) . the findings of focal endovascular granulation tissue and perivascular fibromuscular proliferation in the renal artery were suggestive of a vasculitis - associated change , rather than fmd , in our case . the partial involvement of the circumference and definite inflammatory response seen in our case are unusual for fmd , hence the localized involvement of vasculitis , such as ta , was suspected . in our case , vasculitis can be classified into large - vessel vasculitis including ta , polymyalgia rheumatic , and temporal arteritis . among these large - vessel vasculitis classifications , another possible diagnosis was a secondary change associated with septic emboli ; however , our patient had no specific medical history suggestive of that diagnosis . mri can be helpful because a thickened blood vessel wall is suggestive of the edema and inflammation seen in ta3 ) . the other tools are positron emission tomography ( pet ) scans that are helpful in the diagnosis of inflammation in the blood vessel wall310 ) . therefore , further evaluation such as magnetic resonance angiography and pet may be necessary to confirm a ta diagnosis when a case shows the clinical manifestations associated with ta in the future . many patients with ta have an increased acute phase reactant such as esr or crp . the former is characterized by nonspecific symptoms such as fever , weight loss or fatigue , and increased esr13 ) . half of the patients with ta are undiagnosed before the chronic phase in which symptoms caused by vascular occlusion , such as hypertension , predominante3 ) . total occlusion of the renal artery appeared to be chronic and the esr was within the normal range , therefore our study subject was diagnosed in the chronic phase . renovascular hypertension caused by vasculitis is generally resistant to medical therapy and often requires additional invasive management , such as angioplasty or surgical bypass11 ) . recently , less invasive percutaneous transluminal angioplasty has become the first line therapy in such cases10 ) . if it is unsuccessful or technically impossible , prompt renal artery bypass with the use of a saphenous vein graft with aortic inflow or relief of atypical coarctation with bypass grafting should be performed to lower blood pressure11 ) . nephrectomy was performed in our patient in order to control the blood pressure because the left kidney was nonfunctional according to the dmsa scan . additionally , the mass lesion on ct angiography required pathologic evaluation . there have been reports that patients with ta showed a relapse when attempts are made to taper off the immunosuppressant or after successful angioplasty . close monitoring for clinical findings associated with ta relapse will be necessary in our case . this case suggested that childhood ta with renal artery involvement can present with pres , despite the absence of typical ta symptoms . appropriate diagnosis and immediate control of blood pressure are needed in order to have a good prognosis .
takayasu arteritis ( ta ) is a chronic inflammatory disease of unknown etiology that affects mainly the aorta , main aortic branches , and pulmonary arteries . 
 diverse neurological manifestations of ta have rarely been reported in children . 
 posterior reversible encephalopathy syndrome ( pres ) is a neuroradiological condition that presents with headache , seizure , visual disturbances , and characteristic lesions on imaging . 
 inflammatory condition and severe hypertension in ta can cause pres . 
 we report of a 5-year - old girl with presumed ta who presented with pres and chronic total occlusion in the renal artery . the findings on magnetic resonance imaging suggested pres . 
 left nephrectomy was performed for total occlusion of the left renal artery , and the confirmatory diagnosis of ta was based on the pathologic findings of the renal artery .