Val-Bernal J Fernando, Mayorga Marta, García-Alberdi Eva, Pozueta J Andrés
Department of Anatomical Pathology, Marqués de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain.
Cardiovasc Pathol. 2003 Mar-Apr;12(2):94-7. doi: 10.1016/s1054-8807(02)00157-6.
Churg-Strauss syndrome is a rare disorder characterized by necrotizing vasculitis, granulomas with eosinophilic necrosis, and tissue infiltration by eosinophils. Sudden cardiac death is rarely described in Churg-Strauss syndrome. In this article, we describe a case of Churg-Strauss syndrome with multiorgan involvement manifested as sudden cardiac death. To the best of our knowledge, this form of presentation has not been reported. A 49-year-old woman was found dead in her room. No premonitory complaints had been noted during the days preceding her death. Past medical history did not reveal any relevant illness. At autopsy, multiorganic Churg-Strauss syndrome with prominent cardiac involvement was found. Therefore, this syndrome in the active vasculitic phase may be asymptomatic and may involve predominantly the heart. This variant of the syndrome may be fulminant and present as sudden cardiac death. This form can only be elucidated by autopsy study.
变应性肉芽肿性血管炎是一种罕见疾病，其特征为坏死性血管炎、伴有嗜酸性粒细胞坏死的肉芽肿以及嗜酸性粒细胞的组织浸润。变应性肉芽肿性血管炎中很少描述有心脏性猝死。在本文中，我们描述了一例多器官受累表现为心脏性猝死的变应性肉芽肿性血管炎病例。据我们所知，这种表现形式尚未见报道。一名49岁女性被发现死于其房间内。在其死亡前几天未发现有先兆性不适。既往病史未显示任何相关疾病。尸检时，发现有多器官受累的变应性肉芽肿性血管炎，心脏受累明显。因此，处于活动期血管炎阶段的该综合征可能无症状，且可能主要累及心脏。该综合征的这种变体可能是暴发性的，并表现为心脏性猝死。这种形式只能通过尸检研究来阐明。
