DIFFUSE ALVEOLAR HEMORRHAGE IN A PATIENT WITH PRIMARY ANTIPHOSPHOLIPID SYNDROME: A RARE BUT SERIOUS ENTITY

Abstract

TOPIC: Pulmonary Vascular Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: We present a case of a middle-aged female diagnosed with antiphospholipid syndrome (APS) with biopsy-proven pulmonary capillaritis coming with dyspnea and hemoptysis. CASE PRESENTATION: 66 years female with a history of APS with pulmonary vasculitis, multiple hospitalizations for recurrent pneumonia presented to Emergency Department (ED) with 2 weeks of worsening dyspnea, coughing out of blood clots, & nose bleeds for a day. She denied fever, chills, chest pain, and bleeding from elsewhere. ED vitals includes oxygen saturation of 70% on room air, otherwise afebrile with stable hemodynamics. Physical exam showed diffuse wheezing & crackles over bilateral lungs. Lab revealed pancytopenia requiring blood transfusion. Serology showed persistently positive antiphospholipid antibodies (ACL, Beta 2 Glycoprotein) but negative ANA, dsDNA, C-ANCA, P-ANCA, Anti-GBM antibody, hemolysis labs with normal complement 3 & 4, and ESR/CRP markers. Chest x-ray & CT chest showed diffuse ground-glass opacities. Echo revealed EF 71-75% with pulmonary hypertension. She was admitted to the ICU and started on pulse steroid & broad-spectrum antibiotics. She underwent 4 cycles of plasmapheresis followed by mycophenolate & rituximab for vasculitis flare. After a week, she improved without further need of supplemental oxygen and was discharged with mycophenolate, rituximab & oral steroid. DISCUSSION: Diffuse alveolar hemorrhage (DAH) can be a rare but fatal (21% mortality) manifestation of APS ( a hypercoagulable condition) where antiphospholipid antibodies (aPL) mediated pulmonary endothelial injury causes RBCs to leak into the alveoli [1,2]. The patient may recurrently present with dyspnea, fever, cough, hemoptysis with diffuse ground-glass opacity in chest film and dropping hematocrit [1,2,3]. A thorough history, examination, lab/imaging studies, or sometimes biopsy may be required to diagnose the underlying etiology so that specific treatment can be directed along with supportive care. Serological work-up should include ANCA Abs, ANA, dsDNA, Anti-GBM, complement 3 & 4 levels to identify underlying systemic vasculitis and cardiac echo should exclude heart failure [1]. Patient with this condition has persistently elevated & high titer of aPL antibodies [1,2]. Persistent bloody fluid in bronchoscopy with bronchoalveolar lavage supports DAH [1,3]. Though not recommended routinely, tissue biopsy shows pulmonary capillaritis more often than microvascular thrombosis in APS [1,2]. Respiratory support, maintenance of hemodynamics, temporary avoidance of anticoagulants, exclusion of infection, and initiation of high dose systemic steroid with immunosuppressive agents with or without plasma exchange are key to the management in patients with DAH secondary to capillaritis from underlying autoimmune condition [1,2,3]. CONCLUSIONS: Though rare, DAH should be suspected in patient with APS to direct the appropriate treatment timely. REFERENCE #1: Stoots SA, Lief L, Erkan D. Clinical Insights into Diffuse Alveolar Hemorrhage in Antiphospholipid Syndrome. Curr Rheumatol Rep. 2019;21(10):56. Published 2019 Sep 6. doi:10.1007/s11926-019-0852-7 REFERENCE #2: Deane KD, West SG. Antiphospholipid antibodies as a cause of pulmonary capillaritis and diffuse alveolar hemorrhage: a case series and literature review. Semin Arthritis Rheum. 2005 Dec;35(3):154-65. doi: 10.1016/j.semarthrit.2005.05.006. PMID: 16325656. REFERENCE #3: Gertner E. Diffuse alveolar hemorrhage in the antiphospholipid syndrome: spectrum of disease and treatment. J Rheumatol. 1999 Apr;26(4):805-7. PMID: 10229400. DISCLOSURES: No relevant relationships by Varun tej Gonuguntla, source=Web Response No relevant relationships by Resha Khanal, source=Web Response No relevant relationships by Sharad Oli, source=Web Response No relevant relationships by Shital Oli, source=Web Response No relevant relationships by kiran para, source=Web Response No relevant relationships by Ankur Sinha, source=Web Response No relevant relationships by husnain waseem, source=Web Response