CLOT ME IF YOU CAN: A CASE OF ANTIPHOSPHOLIPID ANTIBODY SYNDROME

Abstract

SESSION TITLE: Monday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Non-bacterial thrombotic endocarditis (NBTE) is a disease characterized by the presence of vegetations on cardiac valves. NBTE has increasingly been recognized as a condition associated with numerous diseases and a potentially life-threatening source of thromboembolism. Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disease defined by thrombotic or obstetrical events that occur in patients with persistent antiphospholipid antibodies. We present a case of NBTE who was found to have valvular regurgitation and thrombotic vegetations, an atypical manifestation of APS. CASE PRESENTATION: A 29 y/o female presented with substernal chest pain, fatigue and shortness of breath with exertion for 2 weeks duration. Review of system was negative. Vital signs were stable. Exam revealed crackles in lung bases, diastolic murmur heard along the left sternal border. She had no significant PMH. She smokes a pack of cigarettes every day. She denied any history of illicit drug use. CXR showed increased pulmonary vascular congestion in lung bases. Labs revealed elevated BNP (>7000). Echo showed normal LVEF, severe AR and moderate MR. TEE confirmed severe AR and severe MR with mass like vegetations and perforation of the aortic and mitral valve. Blood cultures were negative. Work up for culture negative bacterial endocarditis revealed positive antiphospholipid antibody profile with elevated Anti Beta-2 glycoprotein antibody as well as anti-cardiolipin antibody and positive lupus anticoagulant. Anti-smith Ab and anti-ds DNA Ab were negative. Further history revealed patient had 1 still birth and 3 pre-term deliveries, complicated by pre-eclampsia. Based on these findings patient was diagnosed with primary antiphospholipid antibody syndrome. Patient underwent aortic and mitral valve replacements. She clinically improved and was discharged home on coumadin. DISCUSSION: APS can occur either as a primary condition or in association with a systemic autoimmune condition like SLE. Patients can present with a broad spectrum of thrombotic and non thrombotic clinical manifestations. Diagnosis should be suspected when there is occurrence of one or more otherwise unexplained venous or arterial thrombotic events, especially in young patients. Clinical manifestations are thought to be secondary to the effects of antiphospholipid antibody on the pathways of anticoagulation. Our patient presented with atypical manifestations of APS with cardiac involvement and responded well to the valve surgery and anticoagulation. CONCLUSIONS: APS is a rare condition and clinicians should have a high index of suspicion in young patients presenting with thromboembolic manifestations or with signs of NBTE to prevent further life threatening complications. Reference #1: Giannakopoulos, B., and Krilis, S. A. (2013). The pathogenesis of the anti-phospholipid syndrome. N. Engl. J. Med. 368, 1033–1044. doi:10.1056 Reference #2: Crowther MA, Ginsberg JS, Julian J, Denburg J, Hirsh J, Douketis J, Laskin C, Fortin P, Anderson D, Kearon C, Clarke A, Geerts W, Forgie M, Green D, Costantini L, Yacura W, Wilson S, Gent M, Kovacs MJ. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003; 349: 1133–1138. Reference #3: Arnaud L Mathian A Devilliers Het al. Patient-level analysis of five international cohorts further confirms the efficacy of aspirin for the primary prevention of thrombosis in patients with antiphospholipid antibodies. Autoimmun Rev 2014; 14: 192–200. DISCLOSURES: No relevant relationships by Deepthi Gudivada, source=Web Response No relevant relationships by Venkat Rajasurya, source=Web Response