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

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.

• Authors: 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 Lee, Suzanne 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, ARChiVe Investigators Network
• Journal: The Journal of rheumatology (J Rheumatol) Vol. 39 Issue 10 Pg. 2012-20 (Oct 2012) ISSN: 0315-162X [Print] Canada
• PMID: 22859342 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Antirheumatic Agents
• Immunosuppressive Agents
• Cyclophosphamide

Topics

• Adolescent
• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (classification, diagnosis, drug therapy)
• Antirheumatic Agents (therapeutic use)
• Child
• Child, Preschool
• Cyclophosphamide (therapeutic use)
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Male
• Practice Patterns, Physicians'
• Severity of Illness Index