Elderly cases of Churg–Strauss syndrome: Case report and review of Japanese cases

Abstract

Churg-Strauss syndrome (CSS) is a vasculitis usually thought to affect comparatively young individuals, however, elderly cases of CSS are being reported increasingly. We report two elderly cases of CSS and review 123 cases of CSS reported in Japan between 2003 and 2010. The two patients showed typical features of CSS such as asthma, chronic sinusitis, purpura and mononeuritis multiplex. The biopsy specimens demonstrated leukocytoclastic vasculitis with infiltration of eosinophils. Both patients responded well to oral prednisolone. The age of the CSS cases we reviewed ranged from the teens to the 80s, with 47.2% of the patients aged 60 years or older. These patients tended more frequently to have complications such as gastrointestinal involvement, and a higher mortality rate. Elderly cases of CSS are not rare. Correct diagnosis at an earlier stage and careful, long-term follow up are of particular importance in preventing a fatal outcome.