Churg–Strauss syndrome and sudden cardiac death

• Published in: Cardiovascular pathology 2003-03, Vol.12 (2), p.94-97
• Main Authors: Val-Bernal, J.Fernando, Mayorga, Marta, García-Alberdi, Eva, Pozueta, J.Andrés
• Format: Article
• Language: English
• Subjects: Churg-Strauss Syndrome - mortality; Churg-Strauss Syndrome - pathology; Churg–Strauss syndrome; Coronary Vessels - pathology; Death, Sudden, Cardiac - etiology; Death, Sudden, Cardiac - pathology; Female; Heart Septum - pathology; Humans; Middle Aged; Myocardium - pathology; Sudden cardiac death; Vasculitis; Vasculitis - pathology
• ISSN: 1054-8807; 1879-1336
• 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.