CHURG-STRAUSS-SYNDROME IN A DISTRICT HOSPITAL

Published series on Churg-Strauss syndrome (CSS) come from tertiary re ferral centres. We retrospectively studied 23 patients with CSS (18 ma le) seen over 14 years (1982-1995) in a District General Hospital serv ing a population of 500 000. Mean age of onset of vasculitis was 57, 1 0-19 years older than in previous series. The commonest clinical featu res were asthma (22) and eosinophilia >1.5x10(9)/1 (21). Systemic vasc ulitis involving two or more extrapulmonary organs occurred in 22 pati ents, with specific organ involvement of nervous system (18), joints ( 13), muscles (13), lungs (11), skin (11), kidneys (11), heart (10), an d bowel (7). Various classification systems were applied including the Lanham criteria, which were met in 19 patients; the American College of Rheumatology criteria, met in 14; Churg and Strauss criteria, met i n four; and the Chapel Hill Consensus definition, met only in two. ANC A was detected in 10/17 patients where measured. Treatment included co rticosteroids (21), cyclophosphamide (8), azathioprine (9), immunoglob ulin (2), and methotrexate (1). During follow-up six patients died, tw o due to myocardial vasculitis (mean age 52 years), three due to infec tion (mean age 80 years), and one cause unknown. Significant long-term disability was due to asthma in five and neuropathy in six.