BIRMINGHAM VASCULITIS ACTIVITY SCORE (BVAS) IN SYSTEMIC NECROTIZING VASCULITIS

The continuing morbidity of patients with vasculitis, despite the impr oved prognosis with aggressive therapy, underlines the need for accura te disease assessment. We have devised a clinical index of disease act ivity, and evaluated its use in several forms of necrotizing vasculiti s. The weighted score is based on symptoms and signs in nine separate organ systems. Disease features are only scored if they are attributab le to active vasculitis. The Birmingham Vasculitis Activity Score (BVA S) was compared with two other published vasculitis activity scores, w ith the physician's global assessment (PCA), with outcome, and with se rological markers of disease activity. In a cross-sectional study of 2 13 consecutive patients with different forms of vasculitis, all 107 va sculitis patients who were judged completely well on clinical assessme nt had a BVAS score of 0. Twenty-two patients with active vasculitis p rior to treatment had a median score of 7.5 (range 4-30) and 69 with a ctive disease on treatment had a median score of 10 (1-29). Of the 12 who died, median score immediately prior to death was 20.5 (9-30). In a serial prospective study, 30 cases had documented episodes of active disease. During periods of disease activity, the median BVAS values w ere significantly higher than in remission (15 [range 3-32] vs. 0 [0-2 ], p<0.001); the same was true for CRP values (80 [9-361] vs. 13.5 [5- 68], p<0.001). This was not true for erythrocyte sedimentation rate (E SR), haemoglobin (Hb) or von Willebrand factor (VWF). In three patient s with clinically active disease requiring change of therapy, BVAS was elevated whereas the CRP remained < 20; in nine patients in clinical remission, BVAS was < 2 whilst CRP remained > 20. BVAS had low inter-o bserver variability, and agreed significantly with two other indices o f disease activity. This clinical activity index allows precise assess ment of organ involvement in vasculitis compared to a global assessmen t, whilst the cumulative index provides a useful standard by which to assess serological markers and the need for further therapy.