Chaivannacoopt Kai, Flanagan Eliza
Respiratory Department University Hospital Geelong Geelong Australia.
Respirol Case Rep. 2022 Sep 2;10(10):e01033. doi: 10.1002/rcr2.1033. eCollection 2022 Oct.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem disorder, included in the spectrum of the antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides. There are heterogeneous clinical features and a lack of consensus in standardized diagnostic criteria, with an underappreciation of eosinophilic manifestations. There are now reported phenotypical differences between ANCA-positive and negative EGPA, with myocardial involvement, lung infiltrates and gastrointestinal symptoms predominating in ANCA-negative cases. We report a rare presentation of ANCA-negative EGPA in a woman with respiratory, neurological and cardiac involvement, manifesting as a large left ventricular thrombus without significant cardiac dysfunction.
嗜酸性肉芽肿性多血管炎（EGPA）是一种罕见的多系统疾病，属于抗中性粒细胞胞浆抗体（ANCA）相关血管炎范畴。其临床特征多样，标准化诊断标准缺乏共识，嗜酸性表现未得到充分认识。目前有报道称ANCA阳性和阴性EGPA之间存在表型差异，ANCA阴性病例以心肌受累、肺部浸润和胃肠道症状为主。我们报告了一例罕见的ANCA阴性EGPA病例，该病例为一名女性，有呼吸、神经和心脏受累表现，表现为左心室巨大血栓形成但无明显心脏功能障碍。
