Carr W W, Older S A, Rollefson W A, Wellford A L
Rheumatology service, Department of Medicine, Brooke Army Medical Center, Fort Sam Houston, Texas 78234-6200, USA.
J Clin Rheumatol. 1999 Apr;5(2):79-82. doi: 10.1097/00124743-199904000-00008.
Churg-Strauss syndrome (CSS) is a rare vasculitis characterized by the clinical triad of asthma, peripheral eosinophilia, and systemic vasculitis. Pulmonary symptoms occur commonly, but gastrointestinal, renal, cardiac, and musculoskeletal manifestations may also occur. Disease activity and expression can be variable, and progressive organ failure may occur in the absence of other clinical or biochemical evidence of disease activity. We report the case of a 73-year-old man who presented with signs and symptoms of an acute myocardial infarction, eosinophilia, a pulmonary infiltrate, and recent onset asthma. The cardiac catheterization was normal, but an endomyocardial biopsy specimen revealed eosinophilic myositis, granuloma formation, and small vessel vasculitis. A repeat endomyocardial biopsy 1 month after the initiation of high dose prednisone therapy showed no evidence of inflammation and no significant fibrosis. We suggest that a endomyocardial biopsy is a safe and useful tool in the diagnosis and monitoring of therapy in patients with CSS cardiac disease.
变应性肉芽肿性血管炎(CSS)是一种罕见的血管炎,其特征为哮喘、外周血嗜酸性粒细胞增多和系统性血管炎组成的临床三联征。肺部症状常见,但也可能出现胃肠道、肾脏、心脏和肌肉骨骼表现。疾病活动度和表现可能各异,在缺乏疾病活动的其他临床或生化证据时,可能会发生进行性器官衰竭。我们报告一例73岁男性患者,其表现为急性心肌梗死的体征和症状、嗜酸性粒细胞增多、肺部浸润及近期发作的哮喘。心脏导管检查正常,但心内膜活检标本显示嗜酸性肌炎、肉芽肿形成及小血管血管炎。高剂量泼尼松治疗开始1个月后重复心内膜活检,未发现炎症证据及明显纤维化。我们认为心内膜活检是CSS心脏疾病患者诊断及治疗监测中的一种安全且有用的工具。