QJM, Vol 91, Issue 3 219-229, Copyright © 1998 by Oxford University Press
AJ Reid, BD Harrison, RA Watts, SW Watkin, BG McCann and DG Scott
Published series on Churg-Strauss syndrome (CSS) come from tertiary
referral centres. We retrospectively studied 23 patients with CSS (18 male)
seen over 14 years (1982-1995) in a District General Hospital serving a
population of 500,000. Mean age of onset of vasculitis was 57, 10-19 years
older than in previous series. The commonest clinical features were asthma
(22) and eosinophilia > 1.5 x 10(9)/l (21). Systemic vasculitis
involving two or more extrapulmonary organs occurred in 22 patients, with
specific organ involvement of nervous system (18), joints (13), muscles
(13), lungs (11), skin (11), kidneys (11), heart (10), and 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 in four; and the
Chapel Hill Consensus definition, met only in two. ANCA was detected in
10/17 patients where measured. Treatment included corticosteroids (21),
cyclophosphamide (8), azathioprine (9), immunoglobulin (2), and
methotrexate (1). During follow-up six patients died, two due to myocardial
vasculitis (mean age 52 years), three due to infection (mean age 80 years),
and one cause unknown. Significant long-term disability was due to asthma
in five and neuropathy in six.
ORIGINAL PAPERS
Churg-Strauss syndrome in a district hospital
Department of Respiratory Medicine, Norfolk and Norwich Hospital, UK.
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