Bouiller K, Samson M, Eicher J-C, Audia S, Berthier S, Leguy V, Humbert O, Martin L, Lorgis L, Cottin Y, Bonnotte B, Lorcerie B
Department of Internal Medicine and Clinical Immunology, University Hospital of Dijon, Dijon, France.
Intern Med J. 2014 Sep;44(9):928-31. doi: 10.1111/imj.12525.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of systemic vasculitis in which cardiac involvement is frequent and severe, and accounts for half of EGPA-related deaths. ANCA-positive EGPA differs from ANCA-negative EGPA in that the former is significantly associated with renal involvement, peripheral neuropathy and biopsy proven vasculitis, whereas the latter is associated with cardiac involvement. Herein, we report a case of EGPA with myocarditis in a woman, who was successfully treated with steroids and cyclophosphamide. This report highlights the importance of diagnosing cardiac involvement in EGPA early, especially in ANCA-negative patients.
嗜酸性粒细胞性肉芽肿伴多血管炎（EGPA）是一种罕见的系统性血管炎，心脏受累常见且严重，占EGPA相关死亡病例的一半。抗中性粒细胞胞浆抗体（ANCA）阳性的EGPA与ANCA阴性的EGPA不同，前者与肾脏受累、周围神经病变及活检证实的血管炎显著相关，而后者与心脏受累相关。在此，我们报告一例患有心肌炎的女性EGPA病例，该患者接受类固醇和环磷酰胺治疗后痊愈。本报告强调了早期诊断EGPA心脏受累的重要性，尤其是在ANCA阴性的患者中。
