A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis.

R. Suppiah, C. Mukhtyar, O. Flossmann, F. Alberici, B. Baslund, R. Batra, D. Brown, J. Holle, Z. Hruskova, D.R.W. Jayne, A. Judge, M.A. Little, A. Palmisano, C. Stegeman, V. Tesar, A. Vaglio, K. Westman, R. Luqmani

Research output: Contribution to journalArticle (Academic Journal)peer-review

65 Citations (Scopus)


OBJECTIVE: Assessment of disease activity in vasculitis can be achieved using the BVAS, a clinical checklist of relevant symptoms, signs and features of active disease. The aim of this study was to revalidate the BVAS version 3 (BVAS v. 3) in a cohort of patients with systemic vasculitis. METHODS: A total of 238 patients with vasculitis from seven countries in Europe were evaluated at a single time point. Spearman's correlation coefficients were calculated between BVAS v. 3 scores, vasculitis activity index (VAI), physician's global assessment (PGA), the physician's treatment decision, CRP and the vasculitis damage index (VDI) to demonstrate that the BVAS v. 3 measures disease activity. RESULTS: WG (63%), Churg-Strauss syndrome (9%) and microscopic polyangiitis (9%) were the most common diagnoses. The BVAS v. 3 showed convergent validity with the VAI [ρ = 0.82 (95% CI 0.77, 0.85)], PGA [ρ = 0.85 (95% CI 0.81, 0.88)] and the physician's treatment decision [ρ = 0.54 (95% CI 0.44, 0.62)]. There was little or no correlation between BVAS v. 3 and the CRP level [ρ = 0.18 (95% CI 0.05, 0.30)] or with the VDI [ρ = -0.10 (95% CI 0.22, 0.03)]. The inter-observer reliability was very high with an intra-class correlation coefficient (ICC) of 0.996 (95% CI 0.990, 0.998) for the total BVAS v. 3 score. CONCLUSION: The BVAS v. 3 has been evaluated in a large cohort of patients with vasculitis and the important properties of the tool revalidated. This study increases the utility of the BVAS v. 3 in different populations of patients with systemic vasculitis.
Original languageEnglish
Pages (from-to)899-905
Number of pages7
Issue number5
Publication statusPublished - May 2011


  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Churg-Strauss Syndrome
  • Cohort Studies
  • Cross-Sectional Studies
  • Europe
  • Female
  • Granulomatosis with Polyangiitis
  • Humans
  • Male
  • Microscopic Polyangiitis
  • Middle Aged
  • Observer Variation
  • Reproducibility of Results
  • Severity of Illness Index
  • Systemic Vasculitis
  • Young Adult


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