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Diffuse Alveolar Hemorrhage: A Rare Complication of Henoch-Schönlein Purpura Successfully Treated With Rituximab FREE TO VIEW

Tony Abdo, MD; Ali Jabari, MD; Joseph Meharg, MD
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Roger Williams Medical Center/Boston University School of Medicine, Providence, RI

Chest. 2014;146(4_MeetingAbstracts):203A. doi:10.1378/chest.1993431
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SESSION TITLE: Miscellaneous Student/Resident Cases

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 04:30 PM - 05:30 PM

INTRODUCTION: Diffuse alveolar hemorrhage (DAH) occurs in less than 1% of patients with Henoch-Schönlein Purpura (HSP) and carries with it a mortality of 27.8%.1 We report the case of a 60-year-old male with HSP, atrial fibrillation (AFib) and an INR of 8.4, admitted for one day of hemoptysis.

CASE PRESENTATION: Two months prior to admission, skin biopsy demonstrated leukocytoclastic vasculitis (LCV), and the patient was treated with prednisone. One month later, renal biopsy demonstrated HSP nephritis for which he received pulse steroid followed by 60mg of prednisone daily. He now presents with hemoptysis, two teaspoons over the course of the day, and mild dyspnea. He was afebrile with a SaO2 of 96% despite a chest X-ray that showed perihilar airspace disease. INR was 8.4 so vitamin K was given and Coumadin was held. Rapid flu test was negative. Chest CT showed bilateral upper lobe ground glass infiltrates and moderate pleural effusions. Echocardiogram revealed a normal ejection fraction with LVH and mild MR and TR, and there was no response to diuretics. Hemoptysis did not recur. On day 3, with an INR of 1.6, bronchoalveolar lavage (BAL) revealed increasingly hemorrhagic return from the right upper lobe and the left base, with no evidence of airway inflammation or endobronchial lesion. Serology and cultures were unrevealing. A diagnosis of DAH secondary to HSP was made and we decided to treat him with rituximab (RTX). He received his first dose (325mg/m2) on day 7 and was discharged the next day on Prednisone. RTX was continued weekly for 3 additional doses. Ten weeks post treatment, the patient remains asymptomatic.

DISCUSSION: Pulmonary involvement in HSP manifests primarily as DAH. 94% of patients with DAH have kidney involvement1. Hemoptysis occurs in 75%, and lung biopsy may demonstrate LCV (70%) and IgA staining (50%).1 The vasculitis recurred 28% of the time.1 Treatment options include pulse methylprednisolone, cyclophosphamide, azathioprine and cyclosporine.1 A robust literature demonstrates good clinical response with RTX in the treatment of ANCA vasculitis3 and HSP nephritis.2 These outcomes might be due to a decrease in IgA production and hence IgA-immune complexes deposition or from alteration of antibody independent B cell functions.3 The exact mechanism remains unclear. To the best of our knowledge, this is the first report of DAH associated with HSP successfully treated with low dose prednisone and RTX.

CONCLUSIONS: While the precise role of the B cell in DAH associated with HSP awaits further clarification, the clinical response to rituximab in this setting seems promising.

Reference #1: Rajagopala S, et al. Pulmonary hemorrhage in HSP: case report and systemic review of the english litteraure. Semin Arthritis Rheum. 2013 Feb;42(4):391-400

Reference #2: Sala T, et al. Successful Outcome of a Corticodependent HSP Adult with Rituximab. Case Reports in Medicine, Vol.2014, Article ID 619218: Apr 1 2014

Reference #3: Sanz I, Drug Discov Today Ther Strateg. Apr 1, 2009; 6(1): 13-19.

DISCLOSURE: The following authors have nothing to disclose: Tony Abdo, Ali Jabari, Joseph Meharg

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