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

Ravi Suppiah, Chetan Mukhtyar, Oliver Flossmann, Federico Alberici, Bo Baslund, Rajbir Batra, Denise Brown, Julia Holle, Zdenka Hruskova, David R W Jayne, Andrew Judge, Mark A Little, Alessandra Palmisano, Coen Stegeman, Vladimir Tesar, Augusto Vaglio, Kerstin Westman, Raashid Luqmani

Research output: Contribution to journalArticlepeer-review

90 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
Early online date14 Dec 2010
Publication statusPublished - May 2011


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

Cite this