PT - JOURNAL ARTICLE AU - KIMBERLY MORISHITA AU - JAIME GUZMAN AU - PETER CHIRA AU - EYAL MUSCAL AU - ANDREW ZEFT AU - MARISA KLEIN-GITELMAN AU - AMERICA G. URIBE AU - LESLIE ABRAMSON AU - SUSANNE M. BENSELER AU - ANNE EBERHARD AU - KALEO EDE AU - PHILIP J. HASHKES AU - AIMEE O. HERSH AU - GLORIA HIGGINS AU - LISA F. IMUNDO AU - LAWRENCE JUNG AU - SUSAN KIM AU - DANIEL J. KINGSBURY AU - ERICA F. LAWSON AU - TZIELAN LEE AU - SUZANNE C. LI AU - DANIEL J. LOVELL AU - THOMAS MASON AU - DEBORAH McCURDY AU - KATHLEEN M. O’NEIL AU - MARILYNN PUNARO AU - SUZANNE E. RAMSEY AU - ANDREAS REIFF AU - MARGALIT ROSENKRANZ AU - KENNETH N. SCHIKLER AU - ROSIE SCUCCIMARRI AU - NORA G. SINGER AU - ANNE M. STEVENS AU - HEATHER van MATER AU - DAWN M. WAHEZI AU - ANDREW J. WHITE AU - DAVID A. CABRAL TI - Do Adult Disease Severity Subclassifications Predict Use of Cyclophosphamide in Children with ANCA-associated Vasculitis? An Analysis of ARChiVe Study Treatment Decisions AID - 10.3899/jrheum.120299 DP - 2012 Oct 01 TA - The Journal of Rheumatology PG - 2012--2020 VI - 39 IP - 10 4099 - http://www.jrheum.org/content/39/10/2012.short 4100 - http://www.jrheum.org/content/39/10/2012.full SO - J Rheumatol2012 Oct 01; 39 AB - Objective. To determine whether adult disease severity subclassification systems for antineutrophil cytoplasmic antibody-associated vasculitis (AAV) are concordant with the decision to treat pediatric patients with cyclophosphamide (CYC). Methods. We applied the European Vasculitis Study (EUVAS) and Wegener’s Granulomatosis Etanercept Trial (WGET) disease severity subclassification systems to pediatric patients with AAV in A Registry for Childhood Vasculitis (ARChiVe). Modifications were made to the EUVAS and WGET systems to enable their application to this cohort of children. Treatment was categorized into 2 groups, “cyclophosphamide” and “no cyclophosphamide.” Pearson’s chi-square and Kendall’s rank correlation coefficient statistical analyses were used to determine the relationship between disease severity subgroup and treatment at the time of diagnosis. Results. In total, 125 children with AAV were studied. Severity subgroup was associated with treatment group in both the EUVAS (chi-square 45.14, p < 0.001, Kendall’s tau-b 0.601, p < 0.001) and WGET (chi-square 59.33, p < 0.001, Kendall’s tau-b 0.689, p < 0.001) systems; however, 7 children classified by both systems as having less severe disease received CYC, and 6 children classified as having severe disease by both systems did not receive CYC. Conclusion. In this pediatric AAV cohort, the EUVAS and WGET adult severity subclassification systems had strong correlation with physician choice of treatment. However, a proportion of patients received treatment that was not concordant with their assigned severity subclass.