RELAPSES IN PATIENTS WITH A SYSTEMIC VASCULITIS

Treatment with cyclophosphamide and steroids has greatly improved surv ival in patients with systemic necrotizing vasculitis but does not alw ays provide a complete cure. There are as yet few data on the incidenc e, pattern and outcome of relapses in these diseases. We studied relap ses in 150 consecutive patients with an idiopathic necrotizing vasculi tis: 12 with classical polyarteritis (CPAN); 95 with microscopic polya rteritis (MPA); 28 with Wegener's granulomatosis (WG); and 15 with lim ited Wegener's granulomatosis (LWG). The relapse rates and median time to relapse in months were: CPAN, 41.7%/33; MPA, 25.4%/24; WG, 44%/42; LWG, 52%/18. The clinical features of relapse were similar to or more aggressive than those of the original presentation in CPAN and LWG an d included renal disease for the first time, but in MPA and WG, relaps e involved less renal involvement in the majority of cases. Laboratory tests, although often positive at relapse, were unhelpful in its pred iction. The considerable accumulative non-fatal relapse rate contrasts with the very good long-term survival rates, and confirms the importa nce of long-term follow-up in systemic vasculitis.