We report the clinical course of 29 patients with Wegener’s granulomatosis (WG) treated with plasma exchange (PE) in Norway in the period from 1988 to 1999. Median follow-up was 41.5 months. The mean number of exchanges was 8.5 ± 5.8 (range 2–32). Median serum creatinine concentration was 400 µmol/l (range 90–1,356) and 17 patients were dialysis dependent at presentation. Two- and five-year patient survival was 75 and 71%, respectively, and renal (ESRD-free) survival was 74 and 54%, respectively. Seven (50%) of the 14 patients alive in the dialysis group had discontinued dialysis within the first month, and 6 (50%) of 12 patients alive at follow-up had independent renal function. No patients, however, had normal serum creatinine concentration. Median time until development of ESRD for patients presenting with a need for dialysis was ∼32 months. The development of ESRD in 79 patients treated with immunosuppression alone was significantly lower, but when adjusted for serum creatinine there was no difference between patients treated with or without PE. Although a considerable fraction of patients with WG and severe renal involvement regain independent renal function, few will have normal serum creatinine concentration at follow-up, despite the addition of PE as adjunctive therapy.