Wegener's granulomatosis: an analysis of 33 patients seen over a 10-year period

Abstract

This retrospective study examined 33 patients with Wegener's granuloma seen between 1990 and 1999 in the Ayrshire and Arran region of Scotland. There was an estimated annual incidence of 10/million/year. The diagnosis in this series was based on the presence of one or more of the following: a positive histology, a positive c-ANCA or a typical clinical course. Twenty-eight patients were diagnosed based on either a positive histology and/or c-ANCA, whereas the remaining five were diagnosed based on the clinical course. c-ANCA was positive in 79.3% but correlated poorly with disease activity. Nasal biopsies were positive in 40%, whereas 94% of renal biopsies were positive, thereby making nasal biopsies unreliable in the diagnosis. Significantly elevated levels of erythrocyte sedimentation rate (ESR) averaging 77 mm/h were found in 32 patients at diagnosis. This showed fluctuation with disease activity. Thirteen patients died, 12 within 5 years. The best prognostic indicator statistically was age.