Over the last fifteen years, collaborative vasculitis studies in Europe hav
e established the association of antineutrophil cytoplasmic antibodies (ANC
A) with vasculitis and defined its clinical role as a diagnostic tool. More
recently, the European Vasculitis Study Group has developed a consensus ap
proach to the treatment of vasculitis and has aimed to harmonize and optimi
ze 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 vas
culitis, azathioprine is as effective as continued cyclophosphamide for the
maintenance of remission. Other trials are investigating the roles of meth
otrexate, plasma exchange, and pulse cyclophosphamide in acute disease, and
strategies to prevent relapse over the longer term. Parallel studies are d
etermining prognostic markers, evaluating tools for monitoring disease, and
examining the association of ANCA and infection with relapse. The establis
hed infrastructure and accumulated database are well placed to facilitate t
esting of newer therapeutic strategies in the future. Curr Opin Rheumatol 2
001, 13:48-55 (C) 2001 Lippincott Williams & Wilkins, Inc.