Modification and validation of the Birmingham Vasculitis Activity Score (version 3)

Abstract

Background:Comprehensive multisystem clinical assessment using the Birmingham Vasculitis Activity score (BVAS) is widely used in therapeutic studies of systemic vasculitis. Extensive use suggested a need to revise the instrument. The previous version of BVAS has been revised, according to usage and reviewed by an expert committee.Objective:To modify and validate version 3 of the BVAS in patients with systemic vasculitis.Methods:The new version of BVAS was tested in a prospective cross-sectional study of patients with vasculitis.Results:The number of items was reduced from 66 to 56. The subscores for new/worse disease and persistent disease were unified. In 313 patients with systemic vasculitis, BVAS(v.3) correlated with treatment decision (Spearman’s rs = 0.66, 95% CI 0.59 to 0.72), BVAS1 of version 2 (rs = 0.94, 95% CI 0.92 to 0.96), BVAS2 of version 2 in patients with persistent disease (rs = 0.60, 95% CI 0.21 to 0.83), C-reactive protein levels (rs = 0.43, 95% CI 0.31 to 0.54), physician’s global assessment (rs = 0.91, 95% CI 0.89 to 0.93) and vasculitis activity index (rs = 0.88, 95% CI 0.86 to 0.91). The intraclass correlation coefficients for reproducibility and repeatability were 0.96 (95% CI 0.95 to 0.97) and 0.96 (95% CI 0.92 to 0.97), respectively. In 39 patients assessed at diagnosis and again at 3 months, the BVAS(v.3) fell by 17 (95% CI 15 to 19) units (p<0.001, paired t test).Conclusion:BVAS(v.3) demonstrates convergence with BVAS(v.2), treatment decision, physician global assessment of disease activity, vasculitis activity index and C-reactive protein. It is repeatable, reproducible and sensitive to change. The new version of BVAS is validated for assessment of systemic vasculitis.

Prevalence and Responsiveness to Treatment of Lung Abnormalities on Chest Computed Tomography in Patients With Microscopic Polyangiitis: A Multicenter, Longitudinal, Retrospective Study of One Hundred Fifty Consecutive Hospital‐Based Japanese Patients

“…We collected data on disease characteristics, such as concomitant autoimmune diseases, organ involvement, the Disease Extent Index (DEI) (18), the Birmingham Vasculitis Activity Score (BVAS) version 3 (19,20), and the results of laboratory tests including assessment of creatinine, C-reactive protein (CRP), and MPO-ANCA/proteinase 3 (PR3)-ANCA. Organ involvement was determined by the physician using all available information such as clinical symptoms, physical examination, laboratory tests, and other investigations.…”

Hepatotropic Viral Infection Associated Systemic Vasculitides—Hepatitis B Virus Associated Polyarteritis Nodosa and Hepatitis C Virus Associated Cryoglobulinemic Vasculitis

“…The present version (version 3) consists of 56 items with a maximum score of 63. 32 Treatment with corticosteroids and cyclophosphamide help virus persistence and facilitate development of chronic hepatitis and cirrhosis thus cyclophosphamide and prolonged corticosteroids are contraindicated. 6 Both immunosuppression and plasmapheresis due to its central venous access predispose these patients to infections and thus associated mortality.…”

ANCA-associated vasculitis

“…Disease activity scores such as the Birmingham Vasculitis Activity Score (BVAS) can act as a useful aide memoire. 6 BVAS should also be recorded to measure disease activity, but training should be undertaken so that this tool is used appropriately (available at www.bvasvdi.org ).…”