An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients

Arthritis Rheum. 2000 May;43(5):1021-32. doi: 10.1002/1529-0131(200005)43:5<1021::AID-ANR10>3.0.CO;2-J.


Objective: To examine the outcome in 155 consecutive patients with Wegener's granulomatosis (WG) followed up for a median of 7 years.

Methods: Treatment was adapted to the activity and extent of disease, with regular evaluation by an interdisciplinary team accompanied by group education about vasculitis.

Results: The estimated median survival time was 21.7 years (95% confidence interval [95% CI] 15.60-27.86). Twenty-two patients died; 19 deaths were attributable to WG and/or its treatment. Significant predictors of survival at diagnosis were age >50 years (hazard ratio [HR] 5.45, 95% CI 1.97-15.02), kidney involvement with impaired renal function (HR 5.42, 95% CI 1.76-16.68), and lung involvement (HR 3.75, 95% CI 1.26-11.16). At some stage, 142 patients received prednisone and cyclophosphamide (CYC), usually as daily CYC plus mesna as uroprotection, 50 patients received trimethoprim/sulfamethoxazole, and 45 received methotrexate. Complete remission was achieved in 83 of the 155 patients. One or more relapses occurred in 99 patients after either complete or partial remission. CYC-induced cystitis and myelodysplastic syndrome occurred in 17 and 11 patients, respectively. A cumulative dose of 100 gm or more of CYC resulted in a 2-fold greater risk of CYC-related morbidity than with lower CYC doses. Serious infections occurred in 41 patients.

Conclusion: An interdisciplinary approach to the care of 155 WG patients resulted in a median survival of >21 years. Kidney or lung involvement at diagnosis was predictive of a >3-fold higher mortality. Although CYC remains essential in the treatment of WG, it was administered as briefly as possible and under close surveillance to avoid permanent CYC-related morbidity, which can lead to serious therapeutic problems in chronic relapsing WG.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Cohort Studies
  • Cyclophosphamide / adverse effects
  • Cyclophosphamide / therapeutic use
  • Cystitis / chemically induced
  • Female
  • Granulomatosis with Polyangiitis / therapy*
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Long-Term Care
  • Male
  • Methotrexate / therapeutic use
  • Middle Aged
  • Myelodysplastic Syndromes / chemically induced
  • Patient Care Team*
  • Prednisone / therapeutic use
  • Treatment Outcome


  • Immunoglobulins, Intravenous
  • Cyclophosphamide
  • Prednisone
  • Methotrexate