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Cardiogenic Shock: An Unusual Initial Presentation of Churg-Strauss Syndrome

机译:心源性休克:Churg-Strauss综合征的异常表现

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Churg-Strauss syndrome (CSS) is a rare autoimmune condition, characterized by necrotizing extravascular eosinophil rich granulomatous inflammation of the tissues and disseminated small-medium sized vessel vasculitis in a patient with bronchial asthma and tissue eosinophilia. Though pulmonary involvement is the predominant feature of CSS, extra pulmonary involvement, in particular, cardiac involvement, denotes an adverse outcome. Here we report a 50-year-old female who presented with cardiogenic shock due to an acute coronary event as the initial manifestation of CSS. A subsequent coronary angiogram revealed normal epicardial coronaries. She was a patient with bronchial asthma and developed vasculitic rash, bilateral sensory motor polyneuropathy, and migratory peripheral lung field shadows in the background of peripheral eosinophilia during the course of the illness. She was diagnosed as having CSS based on ACR criteria and aggressively treated with immunosuppressants according to her Five-Factor Score and has shown prompt response to therapy. This case report adds to the literature another rare initial presentation of CSS to the existing array of its clinical manifestations.
机译:Churg-Strauss综合征(CSS)是一种罕见的自身免疫性疾病,其特征是将支气管哮喘和组织嗜酸性粒细胞增多症患者的组织中富含血管外嗜酸性粒细胞肉芽肿性炎症坏死和弥散性中小血管血管炎。尽管肺部受累是CSS的主要特征,但额外的肺部受累(尤其是心脏受累)则表示不良结局。在这里,我们报道了一名50岁女性,由于急性冠状动脉事件而出现心源性休克,这是CSS的最初表现。随后的冠状动脉造影显示正常的心外膜冠状动脉。她是一名支气管哮喘患者,在病程中出现周围性嗜酸性粒细胞增多,并发展为血管性皮疹,双侧感觉运动性多发性神经病和周围性肺野阴影。根据ACR标准,她被诊断为患有CSS,并根据她的五因素评分积极地使用了免疫抑制剂治疗,并显示出对治疗的迅速反应。该病例报告向文献添加了CSS的另一种罕见的初始形式,即其现有的临床表现形式。

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