Stone J H, Hoffman G S, Merkel P A, Min Y I, Uhlfelder M L, Hellmann D B, Specks U, Allen N B, Davis J C, Spiera R F, Calabrese L H, Wigley F M, Maiden N, Valente R M, Niles J L, Fye K H, McCune J W, St Clair E W, Luqmani R A
Johns Hopkins University, Baltimore, Maryland, USA.
Arthritis Rheum. 2001 Apr;44(4):912-20. doi: 10.1002/1529-0131(200104)44:4<912::AID-ANR148>3.0.CO;2-5.
To refine and validate the Birmingham Vasculitis Activity Score (BVAS) as a disease-specific activity index for Wegener's granulomatosis (WG).
Sixteen members of the International Network for the Study of the Systemic Vasculitides (INSSYS) revised the BVAS, with 3 goals: to reduce the redundancy of some component items, to enhance its ability to capture important disease manifestations specific to WG, and to streamline the instrument for use in clinical research. We defined the items and weighted them empirically as either minor (e.g., nasal crusting = 1 point) or major (e.g., alveolar hemorrhage = 3 points). We then validated the new, disease-specific BVAS/WG in 2 simulation exercises and a clinical case series that involved 117 patients with WG.
We removed 38 items from the original BVAS, revised 9 items, and added 7 new items. Correlations between the scores on the BVAS/WG and the physician's global assessment (PGA) of disease activity were high, even when patients in remission were excluded. In the clinical case series, Spearman's rank correlation coefficient between the BVAS/WG and the PGA was r = 0.81 (95% confidence interval 0.73-0.87). The interobserver reliability using intraclass (within-case) correlation coefficients in the 2 simulation exercises was r = 0.93 for the BVAS/WG and r = 0.88 for the PGA in the first and r = 0.91 for the BVAS/WG and r = 0.88 for the PGA in the second. There was no significant observer effect in the scoring of the BVAS/WG or the PGA. The discriminant validity of the BVAS/WG was good: r = 0.73 (95% confidence interval 0.43-0.83).
The BVAS/WG is a valid, disease-specific activity index for WG. Tested in simulation exercises and in actual patients, the BVAS/WG correlates well with the PGA, is sensitive to change, and has good inter- and intraobserver reliability. The INSSYS will use the BVAS/WG to assess the primary outcome in a phase II/III trial of etanercept in WG.
完善并验证伯明翰血管炎活动评分（BVAS）作为韦格纳肉芽肿（WG）的疾病特异性活动指数。
国际系统性血管炎研究网络（INSSYS）的16名成员对BVAS进行了修订，有3个目标：减少部分组成项目的冗余，增强其捕捉WG特有重要疾病表现的能力，简化该工具以便用于临床研究。我们定义了各个项目，并根据经验将其权重分为轻微（如鼻结痂 = 1分）或严重（如肺泡出血 = 3分）。然后我们在2个模拟练习和1个涉及117例WG患者的临床病例系列中验证了新的疾病特异性BVAS/WG。
我们从原始BVAS中删除了38个项目，修订了9个项目，并增加了7个新项目。即使排除缓解期患者，BVAS/WG评分与医生对疾病活动的整体评估（PGA）之间的相关性也很高。在临床病例系列中，BVAS/WG与PGA之间的Spearman等级相关系数为r = 0.81（95%置信区间0.73 - 0.87）。在2个模拟练习中，使用组内（病例内）相关系数的观察者间信度，BVAS/WG为r = 0.93，PGA在第一个模拟练习中为r = 0.88，在第二个模拟练习中BVAS/WG为r = 0.91，PGA为r = 0.88。在BVAS/WG或PGA评分中没有显著的观察者效应。BVAS/WG的判别效度良好：r = 0.73（95%置信区间0.43 - 0.83）。
BVAS/WG是一种有效的、针对WG的疾病特异性活动指数。在模拟练习和实际患者中进行测试后，BVAS/WG与PGA相关性良好，对变化敏感，并且具有良好的观察者间和观察者内信度。INSSYS将使用BVAS/WG评估英夫利昔单抗治疗WG的II/III期试验的主要结局。
