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

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 ste...

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Main Authors: Dan Zhu, Yiming Luo, Xiangyuan Liu, Lingyun Zu
Format: Article
Language:English
Published: Wiley 2017-01-01
Series:Case Reports in Rheumatology
Online Access:http://dx.doi.org/10.1155/2017/2908185
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author Dan Zhu
Yiming Luo
Xiangyuan Liu
Lingyun Zu
author_facet Dan Zhu
Yiming Luo
Xiangyuan Liu
Lingyun Zu
author_sort Dan Zhu
collection DOAJ
description 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.
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publishDate 2017-01-01
publisher Wiley
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series Case Reports in Rheumatology
spelling doaj-art-a6dcdb8a315e46949b8ab65ca03de5da2025-02-03T01:21:24ZengWileyCase Reports in Rheumatology2090-68892090-68972017-01-01201710.1155/2017/29081852908185Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular ThrombosisDan Zhu0Yiming Luo1Xiangyuan Liu2Lingyun Zu3Department of Cardiology, Peking University Third Hospital, Key Laboratory of Cardiovascular Molecular Biology and Regulatory Peptides, Ministry of Health, Beijing, ChinaDepartment of Medicine, Mount Sinai St. Luke’s and Mount Sinai West Hospitals, Icahn School of Medicine at Mount Sinai, New York, NY, USADepartment of Rheumatology and Immunology, Peking University Third Hospital, Beijing, ChinaDepartment of Cardiology, Peking University Third Hospital, Key Laboratory of Cardiovascular Molecular Biology and Regulatory Peptides, Ministry of Health, Beijing, ChinaEosinophilic 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.http://dx.doi.org/10.1155/2017/2908185
spellingShingle Dan Zhu
Yiming Luo
Xiangyuan Liu
Lingyun Zu
Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis
Case Reports in Rheumatology
title Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis
title_full Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis
title_fullStr Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis
title_full_unstemmed Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis
title_short Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis
title_sort antiproteinase 3 positive eosinophilic granulomatosis with polyangiitis presenting with heart failure and intraventricular thrombosis
url http://dx.doi.org/10.1155/2017/2908185
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AT yimingluo antiproteinase3positiveeosinophilicgranulomatosiswithpolyangiitispresentingwithheartfailureandintraventricularthrombosis
AT xiangyuanliu antiproteinase3positiveeosinophilicgranulomatosiswithpolyangiitispresentingwithheartfailureandintraventricularthrombosis
AT lingyunzu antiproteinase3positiveeosinophilicgranulomatosiswithpolyangiitispresentingwithheartfailureandintraventricularthrombosis