DOING THE RIGHT THING CAN HARM: DIFFUSE ALVEOLAR HEMORRHAGE SECONDARY TO HYDRALAZINE USE

Abstract

SESSION TITLE: Fellows Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Hydralazine-induced vasculitis results from anti-MPO antibody formation and fibrinoid necrosis, leading to disruption of alveolar basement membrane with resultant hemorrhage.1 Rarely does this vasculitis cause diffuse alveolar hemorrhage (DAH).1 Presented is a case where long-term compliance with hydralazine led to the fatal outcome of DAH. CASE PRESENTATION: A 59 year old male with history of HFpEF, HTN, DM, CKD 4 presented with hemoptysis. He was recently admitted for dyspnea and abdominal pain and was discharged the day prior. He had taken hydralazine 100mg BID for 10 years and hydralazine-induced vasculitis was diagnosed at that time with renal biopsy showing pauci-immune glomerulonephritis. On physical exam, a copious amount of blood was present in the mouth. The patient was intubated and admitted to ICU. Bronchoscopy revealed DAH diagnosed by progressively bloodier bronchial alveolar lavages. Treatment consisted of high dose steroids, plasmapheresis, and rituximab with little improvement. The patient continued to have hemoptysis and episodes of oxygen desaturation requiring multiple bronchoscopies to retrieve blood clots and mucous plugs. Lab studies revealed an ANA with a 1:640 titer, anti-MPO antibody greater than 800 unit/mg, and a positive dsDNA with a 1:20 titer. CT thorax demonstrated diffuse bilateral ground-glass opacities. Cyclophosphamide was started due to severe disease resistance to the previously described treatments. The patient initially had good improvement with cyclophosphamide, however, his ICU course was later complicated by profound neutropenia leading to septic shock with multi-organ failure due invasive aspergillosis and recurrent duodenal ulcer bleeding. On hospital day 25, the patient passed away due to these complications. DISCUSSION: Hydralazine-induced vasculitis is a rare entity, occurring in 10 cases per million.2 Majority of cases occur in African-American females around age 70.2 It commonly presents with fever, rash, and joint pain. Renal failure may occur, but pulmonary complications are extremely rare.3 Our case is amongst only a few cases reported of alveolar hemorrhage attributed to hydralazine. Some patients studied achieved remission, with severe cases amenable to rituximab.2 Our patient had multiple rituximab treatments, along with plasmapheresis, steroids, and cyclophosphamide, but progressed to multi-organ failure and eventual death. CONCLUSIONS: Even with proper treatment, patients may progress to ARDS, as seen in our patient. Early diagnosis is imperative to prevent fatal complications. By sharing our case, we hope to increase awareness and prompt further research into a rare but serious complication of a commonly used drug, as fatality rates have increased despite management with current guidelines. Reference #1: Aeddula NR, Pathireddy S, Ansari A, et al. Hydralazine-associated antineutrophil cytoplasmic antibody vasculitis with pulmonary–renal syndrome. Case Reports 2018;2018:bcr-2018-227161. Reference #2: Timlin H, Liebowitz JE, Jaggi K, Geetha D. Outcomes of hydralazine induced renal vasculitis. Eur J Rheumatol. 2018;5(1):5–8. https://doi.org/10.5152/eurjrheum.2017.17075 Reference #3: Thakur, K. et al. “Hydralazine Induced Vasculitis Manifesting in Alveolar Hemorrhage” Crit Care Med. 2014; vol. 42, https://doi.org/10.1097/01.ccm.0000458649.47284.46. DISCLOSURES: No relevant relationships by Fatima Anjum, source=Web Response No relevant relationships by Alex Diaz, source=Web Response No relevant relationships by Aamna Hassan, source=Web Response No relevant relationships by Javed Iqbal, source=Web Response No relevant relationships by James Kang, source=Web Response No relevant relationships by Hyfaa Mashaal, source=Web Response No relevant relationships by Rezwan Munshi, source=Web Response No relevant relationships by José Russe-Russe, source=Web Response