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

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Morishita, K; Guzman, J; Chira, P; Muscal, E; Zeft, A; Klein-Gitelman, M; Uribe, AG; Abramson, L; Benseler, SM; Eberhard, A; Ede, K; Hashkes, PJ; Hersh, AO; Higgins, G; Imundo, LF; Jung, L; Kim, S; Kingsbury, DJ; Lawson, EF; Lee, T; Li, SC; Lovell, DJ; Mason, T; McCurdy, D; O'Neil, KM; Punaro, M; Ramsey, SE; Reiff, A; Rosenkranz, M; Schikler, KN; Scuccimarri, R; Singer, NG; Stevens, AM; van Mater, H; Wahezi, DM; White, AJ; Cabral, DA; ARChiVe Investigators Network,

Published Date

  • October 2012

Published In

Volume / Issue

  • 39 / 10

Start / End Page

  • 2012 - 2020

PubMed ID

  • 22859342

International Standard Serial Number (ISSN)

  • 0315-162X

Digital Object Identifier (DOI)

  • 10.3899/jrheum.120299


  • eng

Conference Location

  • Canada