Outcome analysis of patients with vasculitis associated with antineutrophil cytoplasmic antibodies

F Brijker, CC Magee, JWC Tervaert, S O'Neill, JJ Walshe*

*Corresponding author for this work

    Research output: Contribution to journalArticleAcademicpeer-review

    26 Citations (Scopus)


    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.

    Original languageEnglish
    Pages (from-to)344-351
    Number of pages8
    Issue number6
    Publication statusPublished - Dec-1999


    • ANCA
    • vasculitis
    • Wegener's granulomatosis
    • microscopic polyangiitis
    • ANCA

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