The Churg-Strauss syndrome. Report and analysis of 20 cases

Churg-Strauss syndrome (CSS) is a disorder characterised by hypereosinophil ia and systemic vasculitis complicating a preexisting asthma. Twenty cases have been studied. Mean duration of asthma before CSS was 8 years, the peri pheral-blood eosinophilia, always > 1 700/muL, went above 5 000/muL in 17 c ases. The clinical manifestations were the following : 20 impairements of g eneral state with fever, 13 peripheral neuropathies, 15 cutaneous injuries, 10 pericardial or myocardial attacks, 10 digestive impairements, 9 muscula r and articular diseases, 7 renal diseases - all of them linked with the va sculitis - and 9 upper respiratory tract involvements. Pleuropulmonary or c ardiac anomalies have been discovered at the chest X-ray in 14 cases. The d iagnosis has been histologicaly confirmed in 15 cases. No clinical or biolo gical or evolutive distinction was noticed between the 15 positive biopsy p atients and the 5 negative ones. During 8,4 (+/- 7, 9 years) the evolution was caracterised by relapses which have always been announced by increasing eosinophilia. Eighteen patients have been successfully treated with cortic oids alone or associated with cyclophosphamid in 9. Survival at 5 years was 85%. Five deaths occured because of CSS (2 because of an unadapted treatme nt). We have to focus the need for an emergency treatment of CSS. The diagn osis can be done by clinical investigation only, before any anatomopatholog ic results.