Diffuse Lung Disease: Student/Resident Case Report Poster - Diffuse Lung Disease |

A Rare Case of Isolated Pauciimmune Pulmonary Capillaritis (IPPC): Managing Recurrent Alveolar Hemorrhage and Concomitant Pulmonary Embolism FREE TO VIEW

Natsu Fukui, MD; Eric Libre, MD; John Paul Verderese, MD; Christopher King, MD
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Inova Fairfax Hospital, Annandale, VA

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

Chest. 2016;150(4_S):523A. doi:10.1016/j.chest.2016.08.537
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SESSION TITLE: Student/Resident Case Report Poster - Diffuse Lung Disease

SESSION TYPE: Student/Resident Case Report Poster

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

INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a clinicopathological syndrome that can rapidly progress into life threatening respiratory failure. Most cases are associated with systemic vasculitides, and management is aimed at diagnosis and treatment of the underlying cause. Less is known about the management of IPPC.

CASE PRESENTATION: A 74-year-old woman first presented to our institution in 2010 with dyspnea and hemoptysis. Chest CT showed diffuse infiltrates (Image 1), and video assisted thoracoscopic lung biopsy revealed pulmonary hemorrhage with focal neutrophilic capillaritis. Extensive work-up (including ANCA panel, ANA, rheumatoid factor, anti-dsDNA, celiac serologies, anti-GBM, anti-cardiolipin, cryoglobulins, vWF) was unrevealing for an underlying cause, suggesting the diagnosis of IPPC. Prednisone and cyclophosphamide were initiated, resulting in symptom improvement, but development of atypical bladder cytology necessitated discontinuation of cyclophosphamide. She was tried on azathioprine then later mycophenolate, but had recurrent DAH after attempts at dose reduction. Her case was further complicated by out of hospital thrombotic events. In 2011, she represented with bilateral PE (Image 2), and an IVC filter was placed as an alternative to anticoagulation which posed a life-threatening bleeding risk. Aspirin 81mg daily was initiated which was changed to every-other-day dosing when she had another DAH flare. Four years later, she had a second out of hospital PE. She was treated with heparin drip and discharged on rivaroxaban, but unfortunately experienced recurrence of DAH. Rivaroxaban was discontinued, high dose prednisone was initiated, and her IVC filter was replaced. She also completed a four-week course of weekly rituximab, and restarted Aspirin 81mg daily. On this therapy she has remained free of both alveolar hemorrhage and pulmonary thrombosis.

DISCUSSION: Only a handful of cases on IPPC have been reported in the literature, with a single case report of successful remission with rituximab. Thus far, an association with IPPC and hypercoagulable disease has not been reported. The exact etiology of our patient’s PE remains unclear. Extensive work up revealed no predisposition for clotting, and ultrasound showed no DVT. Acute thromboembolism is increasingly recognized as a complication of many systemic vasculitides, and it is possible that her disease activity triggered the hypercoagulable state responsible for her thrombotic events.

CONCLUSIONS: Both alveolar hemorrhage and de novo clot formation are possible complications of IPPC. Development of thromboembolism in the setting of DAH presents a significant management dilemma. This case sheds light on how both life-threatening complications can be managed by carefully balancing these risks and suppressing the underlying capillaritis.

Reference #1: Thompson G, Specks U, Cartin-Ceba R. Isolated pauciimmune pulmonary capillaritis successfully treated with rituximab. CHEST Journal. 2015;147(4):e134-e136

DISCLOSURE: The following authors have nothing to disclose: Natsu Fukui, Eric Libre, John Paul Verderese, Christopher King

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