Outcome analysis of patients with vasculitis associated with antineutrophil cytoplasmic antibodies

Clin Nephrol. 1999 Dec;52(6):344-51.


Background: Objective scoring systems of disease activity and disease-associated damage have proven useful in the management of patients with systemic vasculitis.

Patients and methods: We used the recently designed Birmingham vasculitis activity score (BVAS; maximum score 63) and vasculitis damage index (VDI; maximum score 59) to assess initial activity and long-term damage, respectively, in ANCA positive patients from one center over a 3-year period. Thirty-two patients with ANCA vasculitis were identified and analyzed as an historic cohort. The median BVAS for all vasculitis patients at first presentation was 19 (range 6 - 36). Patients with Wegener's granulomatosis had a significantly higher total score and respiratory BVAS score compared to the 15 with microscopic polyangiitis. The majority of patients received standard cyclophosphamide/steroid treatment.

Results: At the end of follow-up (mean 24.9 months), 4 patients had died; all patients had evidence of permanent organ damage. The median total VDI score at last follow-up was 4.0 (range 0-11), with no differences between patients with Wegener's granulomatosis and microscopic polyangiitis. The VDI was not associated with the number of relapses. A high initial BVAS was found to correlate with a later high vasculitis damage index (r = 0.56). Initial renal or respiratory involvement was also associated with longterm damage in the same organ system.

Conclusion: Although mortality from ANCA-associated vasculitis has decreased, morbidity remains a common problem. High early-disease activity may identify patients at high risk of long-term organ damage, allowing more effective individualized therapy. This hypothesis requires validation in a prospective, controlled study.

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Cohort Studies
  • Cyclophosphamide / therapeutic use
  • Female
  • Follow-Up Studies
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Morbidity
  • Outcome Assessment, Health Care
  • Severity of Illness Index
  • Vasculitis / drug therapy
  • Vasculitis / epidemiology*
  • Vasculitis / immunology
  • Vasculitis / mortality


  • Antibodies, Antineutrophil Cytoplasmic
  • Immunosuppressive Agents
  • Cyclophosphamide