RT Journal Article SR Electronic T1 Do Adult Disease Severity Subclassifications Predict Use of Cyclophosphamide in Children with ANCA-associated Vasculitis? An Analysis of ARChiVe Study Treatment Decisions JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP jrheum.120299 DO 10.3899/jrheum.120299 A1 Kimberly Morishita A1 Jaime Guzman A1 Peter Chira A1 Eyal Muscal A1 Andrew Zeft A1 Marisa Klein-Gitelman A1 America G. Uribe A1 Leslie Abramson A1 Susanne M. Benseler A1 Anne Eberhard A1 Kaleo Ede A1 Philip J. Hashkes A1 Aimee O. Hersh A1 Gloria Higgins A1 Lisa F. Imundo A1 Lawrence Jung A1 Susan Kim A1 Daniel J. Kingsbury A1 Erica F. Lawson A1 Tzielan Lee A1 Suzanne C. Li A1 Daniel J. Lovell A1 Thomas Mason A1 Deborah McCurdy A1 Kathleen M. O'Neil A1 Marilynn Punaro A1 Suzanne E. Ramsey A1 Andreas Reiff A1 Margalit Rosenkranz A1 Kenneth N. Schikler A1 Rosie Scuccimarri A1 Nora G. Singer A1 Anne M. Stevens A1 Heather van Mater A1 Dawn M. Wahezi A1 Andrew J. White A1 David A. Cabral YR 2012 UL http://www.jrheum.org/content/early/2012/07/26/jrheum.120299.abstract 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.