PT - JOURNAL ARTICLE AU - KIMBERLY MORISHITA AU - SUZANNE C. LI AU - EYAL MUSCAL AU - STEVEN SPALDING AU - JAIME GUZMAN AU - AMERICA URIBE AU - LESLIE ABRAMSON AU - KEVIN BASZIS AU - SUSANNE BENSELER AU - SUZANNE BOWYER AU - SARAH CAMPILLO AU - PETER CHIRA AU - AIMEE O. HERSH AU - GLORIA HIGGINS AU - ANNE EBERHARD AU - KALEO EDE AU - LISA IMUNDO AU - LAWRENCE JUNG AU - SUSAN KIM AU - DANIEL J. KINGSBURY AU - MARISA KLEIN-GITELMAN AU - ERICA F. LAWSON AU - DANIEL J. LOVELL AU - THOMAS MASON AU - DEBORAH McCURDY AU - KABITA NANDA AU - LORIEN NASSI AU - KATHLEEN M. O’NEIL AU - EGLA RABINOVICH AU - SUZANNE E. RAMSEY AU - ANDREAS REIFF AU - MARGALIT ROSENKRANZ AU - KENNETH SCHIKLER AU - ANNE STEVENS AU - DAWN WAHEZI AU - DAVID A. CABRAL TI - Assessing the Performance of the Birmingham Vasculitis Activity Score at Diagnosis for Children with Antineutrophil Cytoplasmic Antibody-associated Vasculitis in A Registry for Childhood Vasculitis (ARChiVe) AID - 10.3899/jrheum.111030 DP - 2012 May 01 TA - The Journal of Rheumatology PG - 1088--1094 VI - 39 IP - 5 4099 - http://www.jrheum.org/content/39/5/1088.short 4100 - http://www.jrheum.org/content/39/5/1088.full SO - J Rheumatol2012 May 01; 39 AB - Objective. There are no validated tools for measuring disease activity in pediatric vasculitis. The Birmingham Vasculitis Activity Score (BVAS) is a valid disease activity tool in adult vasculitis. Version 3 (BVAS v.3) correlates well with physician’s global assessment (PGA), treatment decision, and C-reactive protein in adults. The utility of BVAS v.3 in pediatric vasculitis is not known. We assessed the association of BVAS v.3 scores with PGA, treatment decision, and erythrocyte sedimentation rate (ESR) at diagnosis in pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods. Children with AAV diagnosed between 2004 and 2010 at all ARChiVe centers were eligible. BVAS v.3 scores were calculated with a standardized online tool (www.vasculitis.org). Spearman’s rank correlation coefficient (rs) was used to test the strength of association between BVAS v.3 and PGA, treatment decision, and ESR. Results. A total of 152 patients were included. The physician diagnosis of these patients was predominantly granulomatosis with polyangiitis (n = 99). The median BVAS v.3 score was 18.0 (range 0–40). The BVAS v.3 correlations were rs = 0.379 (95% CI 0.233 to 0.509) with PGA, rs = 0.521 (95% CI 0.393 to 0.629) with treatment decision, and rs = 0.403 (95% CI 0.253 to 0.533) with ESR. Conclusion. Applied to children with AAV, BVAS v.3 had a weak correlation with PGA and moderate correlation with both ESR and treatment decision. Prospective evaluation of BVAS v.3 and/or pediatric-specific modifications to BVAS v.3 may be required before it can be formalized as a disease activity assessment tool in pediatric AAV.