Longterm outcome of Wegener's granulomatosis in patients with renal disease requiring dialysis

Objective, It is known that renal failure is a poor prognostic marker for s urvival in Wegener's granulomatosis (WG). We investigated the longterm outc ome of patients with WG who have severe renal disease requiring dialysis. Methods. We performed a retrospective analysis of 104 patients with WG foll owed at our institution between 1982 and 1997. Twenty-three patients who re quired dialysis were studied in detail to determine outcomes and factors th at influenced survival and restoration of renal function. Results, Of 23 dialysis dependent patients with WG, 11 died (Group 1), 7 ei ther remained dialysis dependent or received successful renal transplants ( Group 2), and 5 substantially recovered renal function (Group 3). Mean seru m creatinine at the end of a mean followup period of 38.4 months for Group 3 was 1.8 mg/dl. There was no apparent difference between groups in regard to disease profile, e.g., distribution of organ involvement or serum creati nine when renal impairment was first recognized (mean serum creatinine for groups: 1: 3.0 mg/dl; 2: 5.6 mg/dl; 3: 5.5 mg/dl) and peak serum creatinine prior to dialysis (means for groups: 1: 9.5 mg/dl; 2: 10.5 mg/dl; 3. 9.6 m g/dl). Infection secondary to immunosuppression was the leading cause of de ath in Group 1 patients. Conclusion. Because the clinical profile and degree of renal failure, as ju dged by serum creatinine, did not differ among patients who did or did not regain dialysis independent renal function, we recommend aggressive immunos uppressive therapy in all cases of active WG with acute rapidly worsening r enal failure, regardless of the severity of renal impairment.