Churg–Strauss syndrome and sudden cardiac death J. Fernando Val-Bernal a, *, Marta Mayorga a , Eva Garcı ´a-Alberdi a , J. Andre ´s Pozueta b a Department of Anatomical Pathology, Marque ´s de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain b Office of the Medical Examiner, Marque ´s de Valdecilla University Hospital, Medical Faculty, University of Cantabria, Santander, Spain Received 12 April 2002; received in revised form 25 June 2002; accepted 3 September 2002 Abstract 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. D 2003 Elsevier Inc. All rights reserved. Keywords: Churg –Strauss syndrome; Vasculitis; Sudden cardiac death 1. Introduction Churg–Strauss syndrome is a distinctive clinicopatho- logical syndrome characterized by three main histologic features: necrotizing vasculitis, tissue infiltration by eosino- phils, and extravascular (‘‘allergic’’) granulomas [1]. The disorder is considered a systemic vasculitis with multi- organic involvement and diverse presentations. Many patients have severe asthma that precedes evidence of vasculitis, fever attacks, and peripheral blood eosinophilia [2]. However, nonasthmatic cases [3,4] and a limited form [5] of the disorder have been reported. Sudden cardiac death is rarely described in Churg–Strauss syndrome. As far as we are aware, only two cases of sudden cardiac death in Churg–Strauss syndrome have been reported [6,7]. In this article, we describe a case of asymptomatic Churg–Strauss syndrome with multiorganic involvement manifested as sudden cardiac death. To the best of our knowledge, this form of presentation has not been reported in the multi- organic syndrome. 2. Case report A 49-year-old woman was found dead in her room at 9:15 a.m. A week before, she had been under symptomatic treatment for bronchitis. However, according to the mem- bers of her family, no premonitory complaints had been noted during the days preceding her death. Furthermore, she had been seen in stable condition and performed her usual day activities less than 24 h before being found dead. Past medical history did not reveal any relevant illness. A forensic autopsy was performed. 3. Pathology At autopsy, the heart weighed 430 g and had a normal configuration. All its chambers were normal in size. The valves were not thickened. The left ventricle, right ventricle, and septum were 1.5-, 0.5-, and 1.2-cm-thick, respectively. The myocardium was flabby and pale with scattered patches of red and yellow-gray discoloration (Fig. 1). Histological random sections from ventricles and septum walls showed an intense diffuse infiltration by eosinophils, disperse gran- ulomas, and necrotizing vasculitis, including small and epicardial coronary arteries. The eosinophilic infiltrate was 1054-8807/03/$ – see front matter D 2003 Elsevier Inc. All rights reserved. doi:10.1016/S1054-8807(02)00157-6 * Corresponding author. Departamento de Anatomı ´a Patolo ´gica, Hospital Universitario Marque ´s de Valdecilla, Avda. Valdecilla 1, E- 39008 Santander, Spain. Tel.: +34-942-202599; fax: +34-942-201903. E-mail address: apavbj@humv.es (J.F. Val-Bernal). Cardiovascular Pathology 12 (2003) 94 – 97