Objective. To refine and validate the Birmingham Vasculitis Activity Score (BVAS) as a disease-specific activity index for Wegener's granulomatosis (WG).Methods. 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.Results. 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 (withincase) 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).Conclusion. The BVAS/WG is a valid, diseasespecific 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 interand intraobserver reliability. The INSSYS will use the BVAS/WG to assess the primary outcome in a phase II/III trial of etanercept in WG.The investigation of new therapies for the treatment of systemic vasculitis and the organization of

Objective To refine and validate the Birmingham Vasculitis Activity Score (BVAS) as a disease‐specific activity index for Wegener's granulomatosis (WG). Methods 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. Results 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). Conclusion 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.

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Objective. To identify serum ion patterns that distinguish remission from active disease in patients with Wegener's granulomatosis (WG).Methods. Using sera collected in the WG Etanercept Trial, we selected samples from patients who either were undergoing a period of extended disease remission or had recent flares of active WG. Unfractionated samples were randomized into sets for training and testing, such that remission sera and active disease sera could be analyzed without batch bias. Molecular species within the sera were ionized by high-resolution, matrixassisted laser desorption ionization time-of-flight mass spectrometry. We then used a bioinformatics patternrecognition tool to identify optimal combinations of ions. During the training stage, the clinical data (remission versus active disease) were provided in association with the spectral data from each sample. In the testing stage, we performed blinded testing on a previously unexamined set of samples.Results Conclusion. This serum proteomic profiling approach appears to be useful in distinguishing between states of stable clinical remission and active disease.