Diffuse Lung Disease: Fellow Case Report Poster - Diffuse Lung Disease II |

ANCA Vasculitis: Fatal Pulmonary Hemorrhage FREE TO VIEW

Nidhi Aggarwal, MBBS; Anand Kumar Rai, MBBS; Rebecca Weiss, MD; Yizhak Kupfer, MD
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Maimonides Medical Center, Brooklyn, NY

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):504A. doi:10.1016/j.chest.2016.08.518
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SESSION TITLE: Fellow Case Report Poster - Diffuse Lung Disease II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary hemorrhage is a medical emergency with high mortality. Rapid diagnosis and timely initiation of immunosuppressive therapy are key to management.

CASE PRESENTATION: An 83 year old female with history of hypertension was admitted for worsening renal failure. Serology revealed positive P-ANCA (1:320), myeloperoxidase antibody, proteinase-3 antibody (108.8 U) consistent with pauci-immune P-ANCA Vasculitis. Despite treatment with steroids, cyclophosphamide and plasmapheresis, she deteriorated with hemoptysis, dyspnea and respiratory failure. Chest imaging revealed extensive bilateral infiltrates predominantly at the lung bases consistent with pulmonary hemorrhage. By day 8, hemoptysis resolved, she was extubated, and plasmapheresis was discontinued. A few days later she again decompensated requiring resumption of high dose IV steroid and plasmapheresis. She died despite all measures.

DISCUSSION: Diffuse alveolar hemorrhage can be divided into immune and non-immune causes. The most common immune etiology is ANCA associated vasculitis. The typical presentation is hemoptysis, acute anemia, and evidence of diffuse pulmonary infiltrates on imaging. A retrospective study to assess factors to help prognosticate outcome of pulmonary hemorrhage found that mortality rates correlated with a high serum LDH, shock, renal failure, age > 60 years, cardiovascular diseases and need for chronic hemodialysis1. The presence of anti-proteinase-3 antibody is another poor prognostic factor 2. Our patient had poor prognosis due to her age, renal failure, and anti- Proteinase 3 antibodies. She died despite aggressive treatment with high dose steroids, cyclophosphamide and plasmapheresis. She was also treated with plasmapheresis, recommended for severe vasculitic disease in several studies including MEPEX trial3. Despite aggressive management she died.

CONCLUSIONS: This case emphasizes the risk factors that lead to poorer outcomes in patients with ANCA vasculitis associated pulmonary hemorrhage. The prompt identification of these patients can assist in early diagnosis and appropriation treatment. Despite maximal aggressive therapy vasculitis associated pulmonary hemorrhage has high mortality.

Reference #1: de Prost, N et al, Diffuse alveolar haemorrhage, European Respiratory Journal, June 2010, 35:6.

Reference #2: Frankel et al, The pulmonary vasculitides, ATS Journals, Aug. 2012, 186:3.

Reference #3: Jayne DRW et al, Randomised trial of plasma exchange or high dose methyl prednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 2007;18:2180-88.

DISCLOSURE: The following authors have nothing to disclose: Nidhi Aggarwal, Anand Kumar Rai, Rebecca Weiss, Yizhak Kupfer

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