Over the last fifteen years, collaborative
vasculitis studies in Europe have established the association of
antineutrophil cytoplasmic antibodies (
ANCA) with
vasculitis and defined its clinical role as a diagnostic tool. More recently, the European
Vasculitis Study Group has developed a consensus approach to the treatment of
vasculitis and has aimed to harmonize and optimize current
therapy by a series of multicenter randomized controlled trials. The first trial to report,
CYClophosphamide or
AZathioprine As a REMission
therapy for
vasculitis (CYCAZAREM), demonstrated that, for generalized
vasculitis,
azathioprine is as effective as continued
cyclophosphamide for the maintenance of remission. Other trials are investigating the roles of
methotrexate,
plasma exchange, and pulse
cyclophosphamide in
acute disease, and strategies to prevent relapse over the longer term. Parallel studies are determining prognostic markers, evaluating tools for monitoring disease, and examining the association of
ANCA and
infection with relapse. The established infrastructure and accumulated database are well placed to facilitate testing of newer therapeutic strategies in the future.