Do Adult Disease Severity Subclassifications Predict Use of Cyclophosphamide in Children with ANCA-associated Vasculitis? An Analysis of ARChiVe Study Treatment Decisions

作者：Morishita Kimberly; Guzman Jaime; Chira Peter; Muscal Eyal; Zeft Andrew; Klein Gitelman Marisa; Uribe America G; Abramson Leslie; Benseler Susanne M; Eberhard Anne; Ede Kaleo; Hashkes Philip J; Hersh Aimee O; Higgins Gloria; Imundo Lisa F; Jung Lawrence; Kim Susan; King**ury Daniel J; Lawson Erica F; Lee Tzielan; Li Suzanne C; Lovell Daniel J; Mason Thomas; McCurdy Deborah; O' Neil Kathleen M; Punaro Marilynn; Ramsey Suzanne E; Reiff Andreas

来源：Journal of Rheumatology, 2012, 39(10): 2012-2020.

DOI：10.3899/jrheum.120299

• PEDIATRIC VASCULITIS
• ANTINEUTROPRIL CYTOPLASMIC ANTIBODIES
• SEVERITY
• CYCLOPHOSPHAMIDE

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). %26lt;br%26gt;Methods. We applied the European Vasculitis Study (EUVAS) and Wegener%26apos;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, %26quot;cyclophosphamide%26quot; and %26quot;no cyclophosphamide.%26quot; Pearson%26apos;s chi-square and Kendall%26apos;s rank correlation coefficient statistical analyses were used to determine the relationship between disease severity subgroup and treatment at the time of diagnosis. %26lt;br%26gt;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 %26lt; 0.001, Kendall%26apos;s tau-b 0.601, p %26lt; 0.001) and WGET (chi-square 59.33, p %26lt; 0.001, Kendall%26apos;s tau-b 0.689, p %26lt; 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. %26lt;br%26gt;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. (First Release Aug 1 2012; J Rheumatol 2012;39:2012-20; doi:10.3899/jrheum.120299

• 出版日期2012-10