title: Increased sensitivity of the European medicines agency algorithm for classification of childhood granulomatosis with polyangiitis.
abstract: OBJECTIVE
  Granulomatosis with polyangiitis (Wegener's; GPA) and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare in childhood and are sometimes difficult to discriminate. We compared use of adult-derived classification schemes for GPA against validated pediatric criteria in the ARChiVe (A Registry for Childhood Vasculitis e-entry) cohort, a Childhood Arthritis and Rheumatology Research Alliance initiative.
  
  
  METHODS
  Time-of-diagnosis data for children with physician (MD) diagnosis of AAV and unclassified vasculitis (UCV) from 33 US/Canadian centers were analyzed. The European Medicines Agency (EMA) classification algorithm and European League Against Rheumatism/Paediatric Rheumatology International Trials Organisation/Paediatric Rheumatology European Society (EULAR/PRINTO/PRES) and American College of Rheumatology (ACR) criteria for GPA were applied to all patients. Sensitivity and specificity were calculated (MD-diagnosis as reference).
  
  
  RESULTS
  MD-diagnoses for 155 children were 100 GPA, 25 microscopic polyangiitis (MPA), 6 ANCA-positive pauciimmune glomerulonephritis, 3 Churg-Strauss syndrome, and 21 UCV. Of these, 114 had GPA as defined by EMA, 98 by EULAR/PRINTO/PRES, and 87 by ACR. Fourteen patients were identified as GPA by EULAR/PRINTO/PRES but not by ACR; 3 were identified as GPA by ACR but not EULAR/PRINTO/PRES. Using the EMA algorithm, 135 (87%) children were classifiable. The sensitivity of the EMA algorithm, the EULAR/PRINTO/PRES, and ACR criteria for classifying GPA was 90%, 77%, and 69%, respectively, with specificities of 56%, 62%, and 67%. The relatively poor sensitivity of the 2 criteria related to their inability to discriminate patients with MPA.
  
  
  CONCLUSIONS
  EULAR/PRINTO/PRES was more sensitive than ACR criteria in classifying pediatric GPA. Neither classification system has criteria for MPA; therefore usefulness in discriminating patients in ARChiVe was limited. Even when using the most sensitive EMA algorithm, many children remained unclassified.
authors.affiliation: Division of Rheumatology, BC Children's Hospital, Vancouver, BC V6H 3V4, Canada.
authors.forename: América G
authors.initials: AG
authors.lastname: Uribe
authors.forename: Adam M
authors.initials: AM
authors.lastname: Huber
authors.forename: Susan
authors.initials: S
authors.lastname: Kim
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: O'Neil
authors.forename: Dawn M
authors.initials: DM
authors.lastname: Wahezi
authors.forename: Leslie
authors.initials: L
authors.lastname: Abramson
authors.forename: Kevin
authors.initials: K
authors.lastname: Baszis
authors.forename: Susanne M
authors.initials: SM
authors.lastname: Benseler
authors.forename: Suzanne L
authors.initials: SL
authors.lastname: Bowyer
authors.forename: Sarah
authors.initials: S
authors.lastname: Campillo
authors.forename: Peter
authors.initials: P
authors.lastname: Chira
authors.forename: Aimee O
authors.initials: AO
authors.lastname: Hersh
authors.forename: Gloria C
authors.initials: GC
authors.lastname: Higgins
authors.forename: Anne
authors.initials: A
authors.lastname: Eberhard
authors.forename: Kaleo
authors.initials: K
authors.lastname: Ede
authors.forename: Lisa F
authors.initials: LF
authors.lastname: Imundo
authors.forename: Lawrence
authors.initials: L
authors.lastname: Jung
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Kingsbury
authors.forename: Marisa
authors.initials: M
authors.lastname: Klein-Gitelman
authors.forename: Erica F
authors.initials: EF
authors.lastname: Lawson
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: Eyal
authors.initials: E
authors.lastname: Muscal
authors.forename: Lorien
authors.initials: L
authors.lastname: Nassi
authors.forename: Egla
authors.initials: E
authors.lastname: Rabinovich
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: Nora G
authors.initials: NG
authors.lastname: Singer
authors.forename: Steven
authors.initials: S
authors.lastname: Spalding
authors.forename: Anne M
authors.initials: AM
authors.lastname: Stevens
authors.forename: David A
authors.initials: DA
authors.lastname: Cabral
mesh_terms: D000465:Algorithms; D002648:Child; D015267:Churg-Strauss Syndrome / Q000145:classification / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000145:classification* / Q000175:diagnosis*; D006801:Humans; D008297:Male; D055953:Microscopic Polyangiitis / Q000145:classification / Q000175:diagnosis*; D012042:Registries; D012680:Sensitivity and Specificity