A disease-specific activity index for Wegener's granulomatosis

Modification of the Birmingham Vasculitis Activity Score

John H. Stone, Gary S. Hoffman, Peter A. Merkel, Yuan I. Min, Misty L. Uhlfelder, David B Hellmann, Ulrich Specks, Nancy B. Allen, John C. Davis, Robert F. Spiera, Leonard H. Calabrese, Fredrick Wigley, Nicola Maiden, Robert M. Valente, John L. Niles, Kenneth H. Fye, Joseph W. McCune, E. William St.clair, Raashid A. Luqmani

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)912-920
Number of pages9
JournalArthritis and Rheumatism
Volume44
Issue number4
DOIs
StatePublished - 2001
Externally publishedYes

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Granulomatosis with Polyangiitis
Vasculitis
Physicians
Systemic Vasculitis
Exercise
Confidence Intervals
Nonparametric Statistics
Nose

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

Cite this

A disease-specific activity index for Wegener's granulomatosis : Modification of the Birmingham Vasculitis Activity Score. / Stone, John H.; Hoffman, Gary S.; Merkel, Peter A.; Min, Yuan I.; Uhlfelder, Misty L.; Hellmann, David B; Specks, Ulrich; Allen, Nancy B.; Davis, John C.; Spiera, Robert F.; Calabrese, Leonard H.; Wigley, Fredrick; Maiden, Nicola; Valente, Robert M.; Niles, John L.; Fye, Kenneth H.; McCune, Joseph W.; St.clair, E. William; Luqmani, Raashid A.

In: Arthritis and Rheumatism, Vol. 44, No. 4, 2001, p. 912-920.

Research output: Contribution to journalArticle

Stone, JH, Hoffman, GS, Merkel, PA, Min, YI, Uhlfelder, ML, Hellmann, DB, Specks, U, Allen, NB, Davis, JC, Spiera, RF, Calabrese, LH, Wigley, F, Maiden, N, Valente, RM, Niles, JL, Fye, KH, McCune, JW, St.clair, EW & Luqmani, RA 2001, 'A disease-specific activity index for Wegener's granulomatosis: Modification of the Birmingham Vasculitis Activity Score', Arthritis and Rheumatism, vol. 44, no. 4, pp. 912-920. https://doi.org/10.1002/1529-0131(200104)44:4<912::AID-ANR148>3.0.CO;2-5
Stone, John H. ; Hoffman, Gary S. ; Merkel, Peter A. ; Min, Yuan I. ; Uhlfelder, Misty L. ; Hellmann, David B ; Specks, Ulrich ; Allen, Nancy B. ; Davis, John C. ; Spiera, Robert F. ; Calabrese, Leonard H. ; Wigley, Fredrick ; Maiden, Nicola ; Valente, Robert M. ; Niles, John L. ; Fye, Kenneth H. ; McCune, Joseph W. ; St.clair, E. William ; Luqmani, Raashid A. / A disease-specific activity index for Wegener's granulomatosis : Modification of the Birmingham Vasculitis Activity Score. In: Arthritis and Rheumatism. 2001 ; Vol. 44, No. 4. pp. 912-920.
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abstract = "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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T1 - A disease-specific activity index for Wegener's granulomatosis

T2 - Modification of the Birmingham Vasculitis Activity Score

AU - Stone, John H.

AU - Hoffman, Gary S.

AU - Merkel, Peter A.

AU - Min, Yuan I.

AU - Uhlfelder, Misty L.

AU - Hellmann, David B

AU - Specks, Ulrich

AU - Allen, Nancy B.

AU - Davis, John C.

AU - Spiera, Robert F.

AU - Calabrese, Leonard H.

AU - Wigley, Fredrick

AU - Maiden, Nicola

AU - Valente, Robert M.

AU - Niles, John L.

AU - Fye, Kenneth H.

AU - McCune, Joseph W.

AU - St.clair, E. William

AU - Luqmani, Raashid A.

PY - 2001

Y1 - 2001

N2 - 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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