Rare manifestations of Churg-Strauss syndrome with mediastinal and Hilar Lymphadenopathies: Report of an autopsy case

Abstract

Churg-Strauss syndrome (CSS) is a rare systemic disorder that is classically characterized by asthma, tissue and blood eosinophilia, and necrotizing vasculitis along with a granulomatous response to eosinophilic necrosis in its full-blown form. It has a predilection for small- and medium-sized vessels of the lungs, but can virtually affect any organ system, including the heart, skin, gastrointestinal tract, and nervous system. Involvement of the lymph node in CSS, however, is still rare with only a few cases reported in the English literature.1-6 Furthermore, the classic histologic features of CSS, particularly necrotizing vasculitis, are infrequently captured in the modern biopsy or autopsy specimens due to the widespread use of steroids.7 Here, we report the autopsy findings of a 69-year-old man with CSS involving mediastinal and hilar lymph nodes and discuss the histologic features of partially treated CSS. Formal written informed consent was not required with a waiver by the appropriate institutional review board and/or national research ethics committee (FWA00021932).