BACKGROUND: In patients with end-stage renal disease (ESRD) due to Wegener's granulomatosis, a decrease in vasculitis activity after the development of ESRD, as described in other autoimmune diseases, has been postulated. However, up to now no data in a larger group of patients with Wegener's granulomatosis on chronic dialysis have been available. METHODS: We retrospectively analysed the clinical course of 35 patients with Wegener's granulomatosis and ESRD during chronic dialysis treatment. Diagnosis was based on clinical manifestation, antineutrophil cytoplasmic antibodies and/or histology. RESULTS: During a mean follow-up of 43 months (5-113 months), six patients died, three related to treatment toxicity. The patient survival rates (according to Kaplan-Meier calculation) were 93% after 2 years and 79% after 5 years. Twenty-nine relapses of Wegener's granulomatosis occurred in 17 patients (relapse rate 0.24/patient/year); 2/3 of the relapses were seen during treatment with steroids, 1/6 during cyclophosphamide therapy. The relapses were not related to the dialysis membrane used. Remission or partial remission could be achieved in 93% of the relapses. CONCLUSIONS: The survival of patients on chronic dialysis treatment due to Wegener's granulomatosis was comparable to that of other patient groups with ESRD. The relapse rate was not different from that of non-dialysed patients with Wegener's granulomatosis, and this finding underlines the need for a therapeutic strategy to maintain long-term remission in dialysis-dependent patients, too.
BACKGROUND: In patients with end-stage renal disease (ESRD) due to Wegener's granulomatosis, a decrease in vasculitis activity after the development of ESRD, as described in other autoimmune diseases, has been postulated. However, up to now no data in a larger group of patients with Wegener's granulomatosis on chronic dialysis have been available. METHODS: We retrospectively analysed the clinical course of 35 patients with Wegener's granulomatosis and ESRD during chronic dialysis treatment. Diagnosis was based on clinical manifestation, antineutrophil cytoplasmic antibodies and/or histology. RESULTS: During a mean follow-up of 43 months (5-113 months), six patients died, three related to treatment toxicity. The patient survival rates (according to Kaplan-Meier calculation) were 93% after 2 years and 79% after 5 years. Twenty-nine relapses of Wegener's granulomatosis occurred in 17 patients (relapse rate 0.24/patient/year); 2/3 of the relapses were seen during treatment with steroids, 1/6 during cyclophosphamide therapy. The relapses were not related to the dialysis membrane used. Remission or partial remission could be achieved in 93% of the relapses. CONCLUSIONS: The survival of patients on chronic dialysis treatment due to Wegener's granulomatosis was comparable to that of other patient groups with ESRD. The relapse rate was not different from that of non-dialysed patients with Wegener's granulomatosis, and this finding underlines the need for a therapeutic strategy to maintain long-term remission in dialysis-dependent patients, too.
Authors: Sofia Lionaki; Susan L Hogan; Caroline E Jennette; Yichun Hu; Julie B Hamra; J Charles Jennette; Ronald J Falk; Patrick H Nachman Journal: Kidney Int Date: 2009-06-17 Impact factor: 10.612