DIFFUSE ALVEOLAR HEMORRHAGE IN PATIENTS WITH PRIMARY VS SECONDARY ANTIPHOSPHOLIPID ANTIBODY SYNDROME

Abstract

SESSION TITLE: Diffuse Lung Disease SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/10/2018 01:00 pm - 02:00 pm PURPOSE: We sought to determine the differences in clinical presentation, management and outcomes of diffuse alveolar hemorrhage in patients with primary and secondary antiphospholipid antibody syndrome (APS) METHODS: We conducted a retrospective chart review of 31 patients with diffuse alveolar hemorrhage (DAH) concurrent with primary and secondary antiphospholipid syndrome from January 1997 to January 2018. The diagnosis of DAH was achieved by i) bronchoalveolar lavage(BAL) revealing grossly hemorrhagic fluid from multiple lobes or increasingly hemorrhagic fluid return on BAL ii) >20% hemosiderin macrophages in BAL iii) DAH confirmed on surgical lung biopsy or autopsy. We reviewed their clinical presentations, laboratory, radiographic, bronchoscopic and pathologic findings and their clinical course. RESULTS: The baseline characteristics were as follows: median age 55 (range 25-88) years; predominantly male (58%); 19.3% smokers. Patients with primary APS (n = 19) and secondary APS (n =12) had similar demographic and laboratory profiles, with the exceptions of 95% of the patients with primary APS had anticardiolipin antibody (n=18) and 63% of them had one of the subtypes of beta-2 glycoprotein antibody (n=12) whilst 75% of the secondary APS patients had a positive anticardiolipin antibody positive(n=9) and 50% of those with a positive beta-2 glycoprotein antibody(n=6). Amongst those who underwent lung biopsies (n=9), 7 patients with primary APS had capillaritis versus only one of the secondary APS patients had capillaritis.DAH was first presentation in 21% (n=4) in primary APS but only in one case (8%) in secondary APS. The management of initial episode of DAH in secondary APS included: 67% required non-invasive ventilation (n=8) and 67% mechanical ventilation (n=8); 41% plasmapheresis (n=5) and 17 % ECMO (n=2). Only 15% of primary APS patients required non-invasive ventilation (n=3), 31% mechanical ventilation(n=6); 21% needed multiple plasmapheresis (n=4) and no patient required ECMO. Despite aggressive management, similar percentage of patients had recurrence of DAH in both subsets with 68% in primary (n=13) and 67% in secondary (n=8) APS. DAH (n=2) and unevaluated sudden onset of respiratory failure (n=1) was the cause of death in primary APS whereas no patients died of DAH or respiratory failure in secondary APS at our institution. CONCLUSIONS: Anticardiolipin antibody is the most common antibody present in primary APS patients. Capillaritis is seen more commonly in primary APS as compared to secondary APS. More aggressive respiratory support was needed in DAH cases in the setting of secondary APS. CLINICAL IMPLICATIONS: DAH in the setting of APS, especially secondary APS can be severe. Prompt recognition and early aggressive treatment are needed. DISCLOSURES: No relevant relationships by Misbah Baqir, source=Web Response No relevant relationships by Jay Ryu, source=Web Response No relevant relationships by Swathi Sangli, source=Web Response No relevant relationships by Ting Yang, source=Web Response