Assessment of the Birmingham vasculitis activity score in patients with MPO-ANCA-associated vasculitis: sub-analysis from a study by the Japanese Study Group for MPO-ANCA-associated vasculitis.

Abstract

OBJECTIVES: In the study cohort enrolled in a prospective open-label, multicenter trial conducted by the Japanese Study Group for MPO-ANCA-associated vasculitis (JMAAV), we conducted this sub-analysis to establish the validity of the Birminghan vasculitis activity score (BVAS) for Japanese patients with MPO-ANCA-associated vasculitis. METHODS: We recorded the BVAS at the time of diagnosis, at 6weeks after the diagnosis, and at 3, 6, 9, 12, 15 and 18months after the diagnosis in this study. RESULTS: The most frequently involved organs in the patients were the lungs, kidneys and the nervous system. The kidney (BVAS; new/worse 69.2%, persistent 40.4%), general (BVAS; new/worse 67.3%, persistent 53.8%), chest (BVAS; new/worse 36.5%, persistent 46.2%) and nervous system (BVAS; new/worse 38.5%, persistent 25.0%) were the organ systems most frequently involved by the disease at the baseline. The BVAS for new/worse disease decreased immediately after induction therapy, while improvement of the BVAS for persistent disease after therapy differed among the organ systems. CONCLUSIONS: BVAS was demonstrated to be a valuable guide for selection of the optimal treatment. Thus, BVAS was also found to be a useful tool in Japanese patients for the assessment of disease activity and degree of organ damage in patients with MPO-ANCA-associated vasculitis.