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Case Reports in Rheumatology
Volume 2017 (2017), Article ID 2908185, 3 pages
https://doi.org/10.1155/2017/2908185
Case Report

Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis

1Department of Cardiology, Peking University Third Hospital, Key Laboratory of Cardiovascular Molecular Biology and Regulatory Peptides, Ministry of Health, Beijing, China
2Department of Medicine, Mount Sinai St. Luke’s and Mount Sinai West Hospitals, Icahn School of Medicine at Mount Sinai, New York, NY, USA
3Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing, China

Correspondence should be addressed to Lingyun Zu

Received 22 August 2016; Revised 7 November 2016; Accepted 5 December 2016; Published 29 January 2017

Academic Editor: Jamal Mikdashi

Copyright © 2017 Dan Zhu et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis commonly with cardiac complications. We describe a case of anti-PR3 ANCA-positive EGPA complicated by congestive heart failure and intraventricular thrombosis. Interestingly, the thrombus was resolved rapidly with steroid and cyclophosphamide in the setting of interrupted anticoagulation. To the best of our knowledge, we report the first case of anti-PR3 positive EGPA with extensive cardiac involvement. Our patient had overlapping features with previously studied ANCA-positive and ANCA-negative EGPA cases. We also hypothesize that the thrombogenic potential of eosinophils may play a central role in thrombogenesis in EGPA and aggressive immunosuppressive therapy remains the cornerstone of treatment, and the addition of anticoagulation therapy in the setting of thrombus formation and also very high risk of bleeding needs to be considered cautiously.