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

Dan Zhu,Lingyun Zu,Xiangyuan Liu,Yiming Luo

doi:10.1155/2017/2908185

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.

Highlights

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome, is a rare systemic vasculitis of small- and medium-sized vessels, with heart involvement being a common complication
We present a case of a 20-year-old male with antiproteinase 3 (PR3) antineu trophil cytoplasmic antibodies (ANCA) positive EGPA complicated by severe cardiomyopathy and intraventricular thrombosis, which responded robustly to aggressive immunosuppressive therapy with successful thrombolysis in the setting of interrupted anticoagulation therapy
We described a case of a young male who presented with severe cardiomyopathy with intraventricular thrombus, clinical peripheral neuropathy, nasal polyps, pulmonary nodules, hepatitis, eosinophilia, and anti-PR3 ANCA positive

Summary

Introduction

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly named Churg-Strauss syndrome, is a rare systemic vasculitis of small- and medium-sized vessels, with heart involvement being a common complication. We present a case of a 20-year-old male with antiproteinase 3 (PR3) antineu trophil cytoplasmic antibodies (ANCA) positive EGPA complicated by severe cardiomyopathy and intraventricular thrombosis, which responded robustly to aggressive immunosuppressive therapy with successful thrombolysis in the setting of interrupted anticoagulation therapy

Case Presentation

Findings

Discussion