The Birmingham Vasculitis Activity Score as a Measure of Disease Activity in Patients with Giant Cell Arteritis

Abstract

Objective To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS) in the assessment of disease activity in giant cell arteritis (GCA). Methods Patients with GCA enrolled in a prospective, multicenter, longitudinal study with symptoms of active vasculitis during any visit were included. Spearman’s rank correlation was used to explore the association of the BVAS with other measures of disease activity. Results During a mean (SD) follow-up of 2.3 (1.6) years, symptoms of active GCA were present in 236 visits in 136 subjects (100 female, 74%). Median (range) BVAS1 (new/worse symptoms) was 1 (0–10) and median (range) BVAS2 (persistent symptoms) was 0 (0–5). Median (range) physician global assessment (PGA) was 4 (0–9) for disease activity in the past 28 days and 2 (0–9) for activity on the day of the visit. Important ischemic manifestations of active vasculitis not captured by the BVAS included tongue/jaw claudication (27%), upper extremity claudication (15%), lower extremity claudication (5%), carotidynia (7%), ischemic retinopathy (5%). During 25 visits (11%) with active disease, all symptoms of active vasculitis were captured in the “Other” category yet still resulted in a BVAS 1 and BVAS 2 of 0. BVAS1 moderately correlated with PGA for the past 28 days (Spearman’s correlation 0.50) and physician-rated disease activity for the past 28 days (Spearman’s correlation 0.46). Conclusions The BVAS has limited utility in GCA. Patients with active GCA can have a BVAS of 0. Many important ischemic symptoms attributable to active vasculitis are not captured in the composite score.

Associated factors of poor treatment outcomes in patients with giant cell arteritis: clinical implication of large vessel lesions

“…The evaluation of disease activity and vascular damage of LVV is challenging [31][32][33]. Ocular manifestations and large artery complications were common damages in patients with GCA [34].…”

Development of a Core Set of Outcome Measures for Large-vessel Vasculitis: Report from OMERACT 2016

“…In terms of a single tool, criteria used by an NIH group for active disease (8), the Birmingham Vasculitis Activity Score (BVAS)(9), the Disease Extent Index for Takayasu’s arteritis (DEI.Tak) (10), and the Indian Takayasu’s Arteritis Score 2010 (ITAS2010) (11) have been used in clinical research for TAK (12–14). A similar disease-specific tool does not exist for GCA and a recent study lead by investigators within the OMERACT Vasculitis Working Group found BVAS to have limited utility in GCA (15). A combination of clinical symptoms, glucocorticoids dose or duration, and/or BVAS has been used in clinical trials of GCA (13, 16–18).…”

Associated factors of poor treatment outcomes in patients with giant cell arteritis: clinical implication of large vessel lesions

“…The evaluation of disease activity and vascular damage of LVV is challenging (29)(30)(31). Ocular manifestations and large-artery complications were common damage in patients with GCA (32).…”