Wegener's granulomatosis in Finland in 1981-2000: risk of dialysis-dependent renal disease

Abstract

Objective: To determine the risk of renal insufficiency requiring transient or chronic dialysis and its contributing factors in patients diagnosed with Wegener's granulomatosis (WG) between 1981 and 2000 in Finland.

Patients and methods: A retrospective cohort study using hospital discharge registers with a review of hospital case reports. Data were complemented with files from the Finnish Registry for Kidney Diseases. All files were reviewed by 8 October 2005.

Results: A total of 492 patients received a verified diagnosis of WG in 1981-2000. Of these, 84 (17%) needed dialysis by the end of follow-up. Of the 84 dialysed patients, 32 (38%) recovered initially, 32 (38%) needed chronic dialysis (dialysed > 3 months), and 19 (23%) received a kidney transplant. Forty-one (49%) of the dialysed patients were alive at the end of follow-up: 16 with a kidney transplant, 14 on dialysis, and 11 without dialysis. The cumulative rate of developing renal involvement leading to dialysis was 14.6% [95% confidence interval (CI) 11.6-18.2] at 5 years and 29.6% (95% CI 21.5-40.0) at 20 years after onset of WG symptoms. Elevated creatinine levels at presentation were associated with an increased rate of renal insufficiency requiring chronic dialysis. Age, gender, and involvement of any particular organ system had no significant effect.

Conclusions: WG patients are at great risk of developing renal insufficiency, and this risk increases as the disease progresses. Elevated creatinine levels at presentation are associated with an increased risk of dialysis-dependent end-stage renal disease.