Estimation of BVAS in patients with microscopic polyangiitis in Japan

Abstract

The validity of the Birmingham Vasculitis Activity Score (BVAS) as an index of disease activity and a predictor of the prognosis and outcome in patients with MPA has not yet been established in Japan. We conducted a retrospective study of the data of 73 patients with MPA who were followed up for at least 2 years. We divided the patients into two groups according to the BVAS, namely, the high-BVAS group (≥16) and the low-BVAS group (<16), and compared the clinical characteristics. In addition, the distribution of the BVAS items in the patients and the items contributing to the total score in MPA patients were analyzed. Remission was achieved in 85% of patients at 1 month. There were no significant differences in the serum CRP, creatinine (Cre), or MPO-ANCA between the high- and low-BVAS group; however, the survival time was significantly shorter (p = 0.048) and the mortality rate significantly higher in the high-BVAS group (p = 0.04). The items of the BVAS contributing to the total score were motor neuropathy, sensory neuropathy, pulmonary infiltrate, hematuria, proteinuria, Cre ≥5.6 mg/dL, hypertension, scleritis, rise in Cre by ≥30%, and myalgia. BVAS was found to be a useful tool for determining the disease activity and outcome in patients with MPA in Japan. The initial BVAS was also predictive of the mortality and survival time and can also be used as a prognostic tool; therefore, use of the tool may facilitate the selection of appropriate treatment.

Clinical Outcomes of Japanese MPO-ANCA-related Nephritis: Significance of Initial Renal Death for Survival

“…The previous retrospective study, which included 73 Japanese MPA patients, showed that a higher BVAS (over 16 points) was correlated with shorter survival time and higher mortality rate (14). In the present study, we were able to stratify patients at high risk of death and ESRD by BVAS.…”

“…Recently, BVAS has been recognized not only as an evaluation tool for disease activity but also as a prognostic factor for patient survival in vasculitides (6,(12)(13)(14). As of yet, however, BVAS has not been sufficiently established as a prognostic factor for MPO-ANCA-related nephritis.…”

Risk prediction model for mortality in microscopic polyangiitis: multicentre REVEAL cohort study

“…The BVAS is an activity index for AAV, scoring 56 symptoms/signs across nine organ systems encompassing the entire body [12,17]. BVAS has been reported to predict poor prognosis in patients with MPA [18][19][20]. A study involving 55 patients with MPA revealed that BVAS was a useful predictor of survival, with high BVAS scores, age > 60 years, and the presence of interstitial lung disease associated with adverse outcomes [19].…”

“…A study involving 55 patients with MPA revealed that BVAS was a useful predictor of survival, with high BVAS scores, age > 60 years, and the presence of interstitial lung disease associated with adverse outcomes [19]. Similarly, a study focusing on 73 Japanese patients with MPA reported signi cantly shorter survival times and higher mortality rates in a group with BVAS scores ≥ 16 [20]. In line with previous research, our multicentre cohort study in Japan found a signi cant association between BVAS scores of 20 and poor life prognosis in patients with MPA.…”

A retrospective study on the outcomes of MPO-ANCA-associated vasculitis in dialysis-dependent patients

“…BVAS is a universal standard to evaluate vasculitic activity, but its value as a predictor of mortality or relapse is controversial [18][19][20]. As for CY use, indications are different between Japanese guidelines [17] and British Society for Rheumatology (BSR)/British Health Professionals in Rheumatology (BHPR) guidelines [21].…”