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A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS).

Publication ,  Journal Article
Stone, JH; Hoffman, GS; Merkel, PA; Min, YI; Uhlfelder, ML; Hellmann, DB; Specks, U; Allen, NB; Davis, JC; Spiera, RF; Calabrese, LH; Fye, KH ...
Published in: Arthritis Rheum
April 2001

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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Published In

Arthritis Rheum

DOI

ISSN

0004-3591

Publication Date

April 2001

Volume

44

Issue

4

Start / End Page

912 / 920

Location

United States

Related Subject Headings

  • Severity of Illness Index
  • Sensitivity and Specificity
  • Reproducibility of Results
  • Observer Variation
  • Humans
  • Granulomatosis with Polyangiitis
  • Arthritis & Rheumatology
  • 1117 Public Health and Health Services
  • 1107 Immunology
  • 1103 Clinical Sciences
 

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Stone, J. H., Hoffman, G. S., Merkel, P. A., Min, Y. I., Uhlfelder, M. L., Hellmann, D. B., … International Network for the Study of the Systemic Vasculitides (INSSYS), . (2001). A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum, 44(4), 912–920. https://doi.org/10.1002/1529-0131(200104)44:4<912::AID-ANR148>3.0.CO;2-5
Stone, J. H., G. S. Hoffman, P. A. Merkel, Y. I. Min, M. L. Uhlfelder, D. B. Hellmann, U. Specks, et al. “A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS).Arthritis Rheum 44, no. 4 (April 2001): 912–20. https://doi.org/10.1002/1529-0131(200104)44:4<912::AID-ANR148>3.0.CO;2-5.
Stone, J. H., et al. “A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS).Arthritis Rheum, vol. 44, no. 4, Apr. 2001, pp. 912–20. Pubmed, doi:10.1002/1529-0131(200104)44:4<912::AID-ANR148>3.0.CO;2-5.
Stone JH, Hoffman GS, Merkel PA, Min YI, Uhlfelder ML, Hellmann DB, Specks U, Allen NB, Davis JC, Spiera RF, Calabrese LH, Wigley FM, Maiden N, Valente RM, Niles JL, Fye KH, McCune JW, St Clair EW, Luqmani RA, International Network for the Study of the Systemic Vasculitides (INSSYS). A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum. 2001 Apr;44(4):912–920.
Journal cover image

Published In

Arthritis Rheum

DOI

ISSN

0004-3591

Publication Date

April 2001

Volume

44

Issue

4

Start / End Page

912 / 920

Location

United States

Related Subject Headings

  • Severity of Illness Index
  • Sensitivity and Specificity
  • Reproducibility of Results
  • Observer Variation
  • Humans
  • Granulomatosis with Polyangiitis
  • Arthritis & Rheumatology
  • 1117 Public Health and Health Services
  • 1107 Immunology
  • 1103 Clinical Sciences