RT Journal Article SR Electronic T1 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) JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP 1088 OP 1094 DO 10.3899/jrheum.111030 VO 39 IS 5 A1 KIMBERLY MORISHITA A1 SUZANNE C. LI A1 EYAL MUSCAL A1 STEVEN SPALDING A1 JAIME GUZMAN A1 AMERICA URIBE A1 LESLIE ABRAMSON A1 KEVIN BASZIS A1 SUSANNE BENSELER A1 SUZANNE BOWYER A1 SARAH CAMPILLO A1 PETER CHIRA A1 AIMEE O. HERSH A1 GLORIA HIGGINS A1 ANNE EBERHARD A1 KALEO EDE A1 LISA IMUNDO A1 LAWRENCE JUNG A1 SUSAN KIM A1 DANIEL J. KINGSBURY A1 MARISA KLEIN-GITELMAN A1 ERICA F. LAWSON A1 DANIEL J. LOVELL A1 THOMAS MASON A1 DEBORAH McCURDY A1 KABITA NANDA A1 LORIEN NASSI A1 KATHLEEN M. O’NEIL A1 EGLA RABINOVICH A1 SUZANNE E. RAMSEY A1 ANDREAS REIFF A1 MARGALIT ROSENKRANZ A1 KENNETH SCHIKLER A1 ANNE STEVENS A1 DAWN WAHEZI A1 DAVID A. CABRAL YR 2012 UL http://www.jrheum.org/content/39/5/1088.abstract 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.