Long-term survival of patients with Wegener's granulomatosis from the American College of Rheumatology Wegener's Granulomatosis Classification Criteria Cohort

Am J Med. 1996 Aug;101(2):129-34. doi: 10.1016/s0002-9343(96)80066-0.


Objective: To determine survivorship in Wegener's granulomatosis (WG) in a well-defined multicenter cohort.

Methods: Follow-up was obtained for 77 of the 85 patients enrolled in the 1990 American College of Rheumatology vasculitis classification study.

Results: There were 28 deaths (10 females and 18 males) among the 77 patients available for follow-up. Standardized mortality ratios (SMR) were calculated with mortality data from the general population and from this group of patients with WG (an SMR of 1 indicates that expected and observed survival are identical). Overall survivorship among patients with WG was substantially reduced in this cohort (SMR = 4.685 +/- 0.65; for females SMR = 6.814 +/- 1.571; for males SMR = 3.998 +/- 0.69).

Conclusion: The life expectancy of patients with WG is reduced compared with the general population.

Publication types

  • Multicenter Study
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Cause of Death
  • Female
  • Follow-Up Studies
  • Granulomatosis with Polyangiitis / classification
  • Granulomatosis with Polyangiitis / mortality*
  • Humans
  • Life Expectancy
  • Male
  • Proportional Hazards Models
  • Severity of Illness Index
  • Sex Distribution
  • Survival Analysis