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以嗜酸性粒细胞性心肌炎为表现的变应性肉芽肿性血管炎:一项诊断挑战。

Churg-Strauss syndrome presenting with eosinophilic myocarditis: a diagnostic challenge.

作者信息

Correia Ana Sofia, Gonçalves Alexandra, Araújo Vítor, Almeida e Silva João, Pereira José Manuel, Rodrigues Pereira Pedro, Pizarro Manuel, Silva João Carlos, Maciel Maria Júlia

机构信息

Cardiology Department, Centro Hospitalar de São João, Porto, Portugal.

出版信息

Rev Port Cardiol. 2013 Sep;32(9):707-11. doi: 10.1016/j.repc.2012.10.017. Epub 2013 Jul 23.

DOI:10.1016/j.repc.2012.10.017
PMID:23890465
Abstract

Churg-Strauss syndrome (CSS) is an unusual disease that presents as systemic vasculitis and peripheral eosinophilia in patients with an atopic constitution. Cardiac involvement is unusual and often not prominent on initial presentation, but is an important cause of morbidity and mortality in patients with CSS. We report the case of a young woman with severe acute myocarditis. Coronary arteriography demonstrated extensive focal vasculopathy, consistent with coronary vasculitis, and myocardial biopsy showed eosinophilic myocarditis. This presentation led to an initial diagnosis of CSS in this patient and appropriate therapy resulted in a spectacular remission of disease activity.

摘要

变应性肉芽肿性血管炎(CSS)是一种罕见疾病,表现为具有特应性体质患者的系统性血管炎和外周血嗜酸性粒细胞增多。心脏受累较为罕见,在初次就诊时通常不明显,但却是CSS患者发病和死亡的重要原因。我们报告一例患有严重急性心肌炎的年轻女性病例。冠状动脉造影显示广泛的局灶性血管病变,符合冠状动脉血管炎,心肌活检显示嗜酸性粒细胞性心肌炎。这一表现使该患者最初被诊断为CSS,恰当的治疗使疾病活动显著缓解。

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