{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T07:27:45Z","timestamp":1780471665159,"version":"3.54.1"},"reference-count":32,"publisher":"The Journal of Rheumatology","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"published-print":{"date-parts":[[2012,10]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>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).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>We applied the European Vasculitis Study (EUVAS) and Wegener\u2019s 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, \u201ccyclophosphamide\u201d and \u201cno cyclophosphamide.\u201d Pearson\u2019s chi-square and Kendall\u2019s rank correlation coefficient statistical analyses were used to determine the relationship between disease severity subgroup and treatment at the time of diagnosis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>In total, 125 children with AAV were studied. Severity subgroup was associated with treatment group in both the EUVAS (chi-square 45.14, p &lt; 0.001, Kendall\u2019s tau-b 0.601, p &lt; 0.001) and WGET (chi-square 59.33, p &lt; 0.001, Kendall\u2019s tau-b 0.689, p &lt; 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.120299","type":"journal-article","created":{"date-parts":[[2012,8,2]],"date-time":"2012-08-02T05:29:34Z","timestamp":1343885374000},"page":"2012-2020","source":"Crossref","is-referenced-by-count":9,"title":["Do Adult Disease Severity Subclassifications Predict Use of Cyclophosphamide in Children with ANCA-associated Vasculitis? 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