Outcome analysis of patients with vasculitis associated with antineutrophil cytoplasmic antibodies

Citation
F. Brijker et al., Outcome analysis of patients with vasculitis associated with antineutrophil cytoplasmic antibodies, CLIN NEPHR, 52(6), 1999, pp. 344-351
Citations number
25
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Urology & Nephrology","da verificare
Journal title
CLINICAL NEPHROLOGY
ISSN journal
0301-0430 → ACNP
Volume
52
Issue
6
Year of publication
1999
Pages
344 - 351
Database
ISI
SICI code
0301-0430(199912)52:6<344:OAOPWV>2.0.ZU;2-J
Abstract
Background: Objective scoring systems of disease activity and disease-assoc iated 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 i ndex (VDI; maximum score 59) to assess initial activity and long-term damag e, respectively, in ANCA positive patients from one center over a 3-year pe riod. Thirty-two patients with ANCA vasculitis were identified and analyzed as an historic cohort. The median BVAS for all vasculitis patients at firs t presentation was 19 (range 6 - 36). Patients with Wegener's granulomatosi s had a significantly higher total score and respiratory BVAS score compare d to the 15 with microscopic polyangiitis. The majority of patients receive d standard cyclophosphamide/steroid treatment. Results: At the end of follo w-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 granu lomatosis and microscopic polyangiitis. The VDI was not associated with the number of relapses. A high initial BVAS was found to correlate with a late r high vasculitis damage index (r = 0.56). Initial renal or respiratory inv olvement was also associated with longterm damage in the same organ system. Conclusion: Although mortality from ANCA-associated vasculitis has decreas ed, morbidity remains a common problem. High early-disease activity may ide ntify patients at high risk of long-term organ damage, allowing more effect ive individualized therapy. This hypothesis requires validation in a prospe ctive, controlled study.