libman - sacks endocarditis was first described in patients with autoimmune disease and systemic lupus erythematosus ( sle ) . libman - sacks endocarditis is characterized by sterile and verrucous lesions that mostly affect the mitral and aortic valves . in most cases however , patients with significant valvular dysfunction may present with serious complications such as cardiac failure , arrhythmias , and thromboembolic events [ 1 , 2 ] . recently , association of libman - sacks endocarditis with antiphospholipid antibody syndrome ( aps ) has been made . aps is most commonly defined by venous and arterial thrombosis , recurrent pregnancy loss , and thrombocytopenia . while the syndrome can be a primary syndrome , it is usually secondary to sle . catastrophic antiphospholipid syndrome ( caps ) can be a life - threatening presentation of aps and can occur in 1% of patients with aps . we present a rare case of a young female patient with lupus - negative libman - sacks endocarditis complicated by caps . a 22-year - old caucasian female with no past medical history presented to the emergency department after being found by her boyfriend unconscious at home . the mother reported that the patient had complaints of generalized malaise and fatigue over the last 2 months , but otherwise she was healthy . family history was significant for hashimoto s thyroiditis in the mother and celiac disease in the father . patient had a two pack year history of smoking and had recently quit 3 months ago . she was an occasional alcohol and marijuana user but did not use any other recreational drugs . on presentation to the emergency department , blood pressure was 90/61 mm hg , heart rate was 127 beats per minute , temperature was 38.9 c , respiratory rate was 28 breaths per minute and saturation was 87% on room air . the patient was subsequently intubated for airway protection . on physical exam , pupils were equal and reactive , 2/6 holosystolic murmur was heard on the left lower sternal border , and pulses were diminished throughout . , there were no spontaneous movements of left upper extremity and minimal movements of the left lower extremity . given the altered mental status , ct of head was obtained which showed extensive multifocal areas of hypoattenuation throughout the bilateral frontal , parietal , occipital and right temporal lobes consistent with multifocal infarction concerning for cardioembolic etiology ( fig . 1 ) . ct of head without contrast showing extensive multifocal areas of hypoattentuation throughout the bilateral frontal , parietal , occipital , and right > left temporal lobes . given the fever , leukocytosis and suspected cardioembolic phenomenon causing infarctions in the brain , there was concern for bacterial endocarditis . hence , transesophageal echocardiogram ( tee ) was performed which showed a 1 cm vegetation at the anterior mitral leaflet ( fig . the patient was started on broad spectrum antibiotics , vancomycin , and ceftriaxone for presumed bacterial endocarditis with systemic emboli . however , blood cultures obtained from two different sites ( peripheral and antecubital ) prior to administration of antibiotics remained negative throughout the hospital stay . 2d transesophageal ultrasound . image on the left shows a thickened mitral valve with a 1 cm vegetation that can be seen on the anterior mitral leaflet . image on the right is a four - chamber color flow doppler view showing biventricular dilatation , severe left ventricular dysfunction . for orientation purposes , thus , hypercoagulable workup was not performed with presumption of false positive findings in the setting of critical illness along with a vegetation that was showering emboli . on day 8 , metabolic laboratory abnormalities improved but patient s neurological status worsened and she no longer opened her eyes spontaneously . that she had multifocal infarctions based on her initial head ct scan was already known . although her initial head ct had shown multiple infarctions , her abrupt neurological decline was unclear . it showed increased signal on dwi throughout bilateral frontal , parietal , and occipital lobes consistent with new / ongoing acute infarcts ( fig . images on the top show increased signal on diffusion weighted imaging ( dwi ) throughout the bilateral frontal , parietal , and occipital lobes . images on the bottom show a corresponding decreased signal intensity on apparent diffusion coefficient that is consistent with acute abnormal restricted diffusion . , patient was found to have a left upper extremity arterial thrombus requiring emergent thrombectomy . heparin - induced thrombocytopenia was on the differential given the drop in platelets while the patient was on heparin . after a palliative care meeting with the family , decision was made to withdraw care and the patient expired . autopsy findings were the following : ischemic necrosis of most organs including the liver , lungs , and adrenal glands ; cardiomegaly with atrial and ventricular thrombi , mitral vegetation with pericardial effusion ; right lung hemorrhage infarct with vascular emboli ; hepatomegaly with congestion , necrosis ; kidneys with extensive necrosis and vascular emboli ; mitral valve vegetation , 10 cm in size , had lots of necrosis and the mitral valve tissue showed focal necrosis without the presence of bacterial or fungal organisms ; myocardium was normal without lymphocytic infiltrates or granulomas ; clusters of fibrinous thrombi with lymphoid myeloid cells adjacent to the myocardial tissue possibly related to extensive vegetations . these findings were consistent with non - bacterial thrombotic endocarditis ( figs . 4 - 8 ) . high power pathology slide of the liver showing lots of steatosis , congestion , and necrosis .   low power pathology slide of the lung showing emboli and necrotic tissue . characteristic laboratory abnormalities in aps include elevated levels of antibodies against the membrane phospholipids and/or their associated plasma proteins . laboratory criteria for diagnosis of aps involve the presence of one or more antiphospholipid antibodies measured on two or more occasions at least 12 weeks apart . these laboratory findings along with clinical criteria of vascular thrombosis or pregnancy mortality are used for the diagnosis of aps . aps can occur as a primary disorder or secondary to an underlying disease such as sle or other systemic autoimmune diseases . caps is the most severe manifestation of aps and tends to occur in 1% of patients with aps . it is defined as thrombosis of at least three different organs systems over a short period of time with histopathologic evidence of ischemia , vessel occlusions and high titers of antiphospholipid antibodies . diagnosis of caps requires the following manifestations : involvement of three organs or systems , development of symptoms within less than 1 week , confirmation by histopathology of small vessel occlusion in at least one organ tissue , and the presence of antiphospholipid antibodies . our patient met the criteria for caps given the autopsy findings . in our case , it was important to differentiate caps from other fatal but similar presentation . the differentiation between hemolytic uremic syndrome ( hus ) , thrombotic thrombocytopenic purpura ( ttp ) and caps is sometimes difficult . while thrombocytopenia and schistocytes are marked in hus / ttp , they are usually absent in caps . activated partial thromboplastin time is usually normal in hus / tts , but it may be elevated in caps in the presence of lupus anticoagulant . caps is usually triggered by a precipitating factor , most likely an infection in younger patients , and malignancy in older patients . any organ can be affected . however , the most common organs affected are kidneys , lungs , and the brain . on the other hand , patients with lupus tend to have more cardiac and brain involvement . finally , management of caps is complex because as there are numerous thrombotic events , the clinical presentation can be difficult to differentiate from that of disseminated intravascular coagulation , and the optimal treatment regimen is unknown . anticoagulation , corticosteroids , plasmapheresis , and intravenous immunoglobulin ( ivig ) are most commonly used in the management of these patients . other reported management options for caps patients include fibrinolytics , prostacyclin , defibrotide , danazol , cyclophosphamide , and azathioprine . looking back in retrospect , we could have treated our patient with either immunosuppression and/or plasmapheresis or steroids . libman - sacks endocarditis , or nbte , was first described in 1924 by libman and sacks . it is characterized by sterile vegetations deposited on heart valves , often favoring the left side of the heart . endothelial damage in the setting of a hypercoagulable state is thought to be a precipitating factor in nbte . other inflammatory states such as antiphospholipid syndrome , sepsis , and rheumatoid arthritis can also be settings for nbte . meanwhile , inflammatory changes can involve all layers of the heart in sle patients with libman - sacks being the most common valvular abnormality . valvular abnormalities have been seen in up to 35% of patients with sle and up to 48% in lupus patients with aps . libman - sacks endocarditis is often times diagnosed on autopsy as symptoms present later in the disease progression . patients may also present with cardiac failure from valvular dysfunction along with generalized symptoms of fatigue , fevers , night sweats , and weight loss . our patient also had diffuse systemic emboli as evidenced on ct of head and mri of brain as well as autopsy findings . caps can be a life - threatening presentation of aps and high clinical index of suspicion is necessary to guide the diagnosis . high clinical suspicion is required and early treatment should be instituted to potentially reverse this highly morbid condition .
libman - sacks endocarditis is characterized by sterile and verrucous lesions that predominantly affect the aortic and mitral valves . in most cases 
 , patients do not have significant valvular dysfunction . 
 however , patients with significant valvular dysfunction may present with serious complications such as cardiac failure , arrhythmias , and thromboembolic events . 
 recently , association of libman - sacks endocarditis with antiphospholipid antibody syndrome ( aps ) has been made . 
 aps is most commonly defined by venous and arterial thrombosis , recurrent pregnancy loss , and thrombocytopenia . while the syndrome can be a primary syndrome , it is usually secondary to systemic lupus erythematosus . 
 catastrophic antiphospholipid syndrome ( caps ) can be a life - threatening presentation of aps and can occur in 1% of patients with antiphospholipid syndrome . 
 we present a very rare case of a young female patient with lupus - negative libman - sacks endocarditis complicated by caps .