SURVIVAL AND VASCULITIS ACTIVITY IN PATIENTS WITH END-STAGE RENAL-DISEASE DUE TO WEGENERS-GRANULOMATOSIS

Background. In patients with end-stage renal disease (ESRD) due to Weg ener's granulomatosis, a decrease in vasculitis activity after the dev elopment of ESRD, as described in other autoimmune diseases, has been postulated. However, up to now no data in a larger group of patients w ith Wegener's granulomatosis on chronic dialysis have been available. Methods. We retrospectively analysed the clinical course of 35 patient s with Wegener's granulomatosis and ESRD during chronic dialysis treat ment. Diagnosis was based on clinical manifestation, antineutrophil cy toplasmic antibodies and/or histology. Results. During a mean follow-u p of 43 months (5-113 months), six patients died, three related to tre atment toxicity. The patient survival rates (according to Kaplan-Meier calculation) were 93% after 2 years and 79% after 5 years. Twenty-nin e relapses of Wegener's granulomatosis occurred in 17 patients (relaps e rate 0.24/patient/year); 2/3 of the relapses were seen during treatm ent with steroids, 1/6 during cyclophosphamide therapy. The relapses w ere not related to the dialysis membrane used. Remission or partial re mission could be achieved in 93% of the relapses. Conclusions. The sur vival of patients on chronic dialysis treatment due to Wegener's granu lomatosis was comparable to that of other patient groups with ESRD. Th e relapse rate was not different from that of non-dialysed patients wi th Wegener's granulomatosis, and this finding underlines the need for a therapeutic strategy to maintain long-term remission in dialysis-dep endent patients, too.