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

Morishita, K.; Guzman, J.; Chira, P.; Muscal, E.; Zeft, A.; Klein-Gitelman, M.; Uribe, A. G.; Abramson, L.; Benseler, S. M.; Eberhard, A.; Ede, K.; Hashkes, P. J.; Hersh, A. O.; Higgins, G.; Imundo, L. F.; Jung, L.; Kim, S.; Kingsbury, D. J.; Lawson, E. F.; Lee, T.; Li, S. C.; Lovell, D. J.; Mason, T.; McCurdy, D.; O'Neil, K. M.; Punaro, M.; Ramsey, S. E.; Reiff, A.; Rosenkranz, M.; Schikler, K. N.; Scuccimarri, R.; Singer, N. G.; Stevens, A. M.; van Mater, H.; Wahezi, D. M.; White, A. J.; Cabral, D. A.

J Rheumatol. 2012 Aug 4; 39(10):2012-2020


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.

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