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.