title: 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.
  
  
  CONCLUSIONS
  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.affiliation: British Columbia Children's Hospital, Vancouver, BC, Canada.
authors.forename: Kimberly
authors.initials: K
authors.lastname: Morishita
authors.forename: Jaime
authors.initials: J
authors.lastname: Guzman
authors.forename: Peter
authors.initials: P
authors.lastname: Chira
authors.forename: Eyal
authors.initials: E
authors.lastname: Muscal
authors.forename: Andrew
authors.initials: A
authors.lastname: Zeft
authors.forename: Marisa
authors.initials: M
authors.lastname: Klein-Gitelman
authors.forename: America G
authors.initials: AG
authors.lastname: Uribe
authors.forename: Leslie
authors.initials: L
authors.lastname: Abramson
authors.forename: Susanne M
authors.initials: SM
authors.lastname: Benseler
authors.forename: Anne
authors.initials: A
authors.lastname: Eberhard
authors.forename: Kaleo
authors.initials: K
authors.lastname: Ede
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Hashkes
authors.forename: Aimee O
authors.initials: AO
authors.lastname: Hersh
authors.forename: Gloria
authors.initials: G
authors.lastname: Higgins
authors.forename: Lisa F
authors.initials: LF
authors.lastname: Imundo
authors.forename: Lawrence
authors.initials: L
authors.lastname: Jung
authors.forename: Susan
authors.initials: S
authors.lastname: Kim
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Kingsbury
authors.forename: Erica F
authors.initials: EF
authors.lastname: Lawson
authors.forename: Tzielan
authors.initials: T
authors.lastname: Lee
authors.forename: Suzanne C
authors.initials: SC
authors.lastname: Li
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Lovell
authors.forename: Thomas
authors.initials: T
authors.lastname: Mason
authors.forename: Deborah
authors.initials: D
authors.lastname: McCurdy
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: O'Neil
authors.forename: Marilynn
authors.initials: M
authors.lastname: Punaro
authors.forename: Suzanne E
authors.initials: SE
authors.lastname: Ramsey
authors.forename: Andreas
authors.initials: A
authors.lastname: Reiff
authors.forename: Margalit
authors.initials: M
authors.lastname: Rosenkranz
authors.forename: Kenneth N
authors.initials: KN
authors.lastname: Schikler
authors.forename: Rosie
authors.initials: R
authors.lastname: Scuccimarri
authors.forename: Nora G
authors.initials: NG
authors.lastname: Singer
authors.forename: Anne M
authors.initials: AM
authors.lastname: Stevens
authors.forename: Heather
authors.initials: H
authors.lastname: van Mater
authors.forename: Dawn M
authors.initials: DM
authors.lastname: Wahezi
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: White
authors.forename: David A
authors.initials: DA
authors.lastname: Cabral
mesh_terms: D000293:Adolescent; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D010818:Practice Patterns, Physicians'; D012720:Severity of Illness Index
chemical_list: D018501:Antirheumatic Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide