Do adult disease severity subclassifications predict use of cyclophosphamide in children with ANCA-associated vasculitis? An analysis of ARChiVe study treatment decisions

Kimberly Morishita, Jaime Guzman, Peter Chira, Eyal Muscal, Andrew Zeft, Marisa Klein-Gitelman, America G. Uribe, Leslie Abramson, Susanne M. Benseler, Anne Eberhard, Kaleo Ede, Philip J. Hashkes, Aimee O. Hersh, Gloria Higgins, Lisa F. Imundo, Lawrence Jung, Susan Kim, Daniel J. Kingsbury, Erica F. Lawson, Tzielan LeeSuzanne C. Li, Daniel J. Lovell, Thomas Mason, Deborah McCurdy, Kathleen M. O'Neil, Marilynn Punaro, Suzanne E. Ramsey, Andreas Reiff, Margalit Rosenkranz, Kenneth N. Schikler, Rosie Scuccimarri, Nora G. Singer, Anne M. Stevens, Heather Van Mater, Dawn M. Wahezi, Andrew J. White, David A. Cabral

Research output: Contribution to journalArticle

5 Scopus citations

Abstract

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. The Journal of Rheumatology

Original languageEnglish (US)
Pages (from-to)2012-2020
Number of pages9
JournalJournal of Rheumatology
Volume39
Issue number10
DOIs
StatePublished - Oct 2012

Keywords

  • Antineutrophil cytoplasmic antibodies
  • Cyclophosphamide
  • Pediatric vasculitis
  • Severity

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology

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    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., ... Cabral, D. A. (2012). Do adult disease severity subclassifications predict use of cyclophosphamide in children with ANCA-associated vasculitis? An analysis of ARChiVe study treatment decisions. Journal of Rheumatology, 39(10), 2012-2020. https://doi.org/10.3899/jrheum.120299