title: Classification, presentation, and initial treatment of Wegener's granulomatosis in childhood.
abstract: OBJECTIVE
  To compare the criteria for Wegener's granulomatosis (WG) of the American College of Rheumatology (ACR) with those of the European League Against Rheumatism/Pediatric Rheumatology European Society (EULAR/PRES) in a cohort of children with WG and other antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs), and to describe the interval to diagnosis, presenting features, and initial treatment for WG.
  
  
  METHODS
  Eligible patients had been diagnosed by site rheumatologists (termed the "MD diagnosis") since 2004. This diagnosis was used as a reference standard for sensitivity and specificity testing of the 2 WG classification criteria. Descriptive analyses were confined to ACR-classified WG patients.
  
  
  RESULTS
  MD diagnoses of 117 patients (82 of whom were female) were WG (n = 76), microscopic polyangiitis (n = 17), ANCA-positive pauci-immune glomerulonephritis (n = 5), Churg-Strauss syndrome (n = 2), and unclassified vasculitis (n = 17). The sensitivities of the ACR and EULAR/PRES classification criteria for WG among the spectrum of AAVs were 68.4% and 73.6%, respectively, and the specificities were 68.3% and 73.2%, respectively. Two more children were identified as having WG by the EULAR/PRES criteria than by the ACR criteria. For the 65 ACR-classified WG patients, the median age at diagnosis was 14.2 years (range 4-17 years), and the median interval from symptom onset to diagnosis was 2.7 months (range 0-49 months). The most frequent presenting features by organ system were constitutional (89.2%), pulmonary (80.0%), ear, nose, and throat (80.0%), and renal (75.4%). Fifty-four patients (83.1%) commenced treatment with the combination of corticosteroids and cyclophosphamide, with widely varying regimens; the remainder received methotrexate alone (n = 1), corticosteroids alone (n = 4), or a combination (n = 6).
  
  
  CONCLUSIONS
  The EULAR/PRES criteria minimally improved diagnostic sensitivity and specificity for WG among a narrow spectrum of children with AAVs. Diagnostic delays may result from poor characterization of childhood WG. Initial therapy varied considerably among participating centers.
authors.affiliation: Division of Rheumatology, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. dcabral@cw.bc.ca
authors.forename: David A
authors.initials: DA
authors.lastname: Cabral
authors.forename: América G
authors.initials: AG
authors.lastname: Uribe
authors.forename: Susanne
authors.initials: S
authors.lastname: Benseler
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: O'Neil
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Hashkes
authors.forename: Gloria
authors.initials: G
authors.lastname: Higgins
authors.forename: Andrew S
authors.initials: AS
authors.lastname: Zeft
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Lovell
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Kingsbury
authors.forename: Anne
authors.initials: A
authors.lastname: Stevens
authors.forename: Deborah
authors.initials: D
authors.lastname: McCurdy
authors.forename: Peter
authors.initials: P
authors.lastname: Chira
authors.forename: Leslie
authors.initials: L
authors.lastname: Abramson
authors.forename: Thaschawee
authors.initials: T
authors.lastname: Arkachaisri
authors.forename: Sarah
authors.initials: S
authors.lastname: Campillo
authors.forename: Anne
authors.initials: A
authors.lastname: Eberhard
authors.forename: Aimee O
authors.initials: AO
authors.lastname: Hersh
authors.forename: Adam M
authors.initials: AM
authors.lastname: Huber
authors.forename: Susan
authors.initials: S
authors.lastname: Kim
authors.forename: Marisa
authors.initials: M
authors.lastname: Klein-Gitelman
authors.forename: Deborah M
authors.initials: DM
authors.lastname: Levy
authors.forename: Suzanne C
authors.initials: SC
authors.lastname: Li
authors.forename: Thomas
authors.initials: T
authors.lastname: Mason
authors.forename: Esi Morgan
authors.initials: EM
authors.lastname: Dewitt
authors.forename: Eyal
authors.initials: E
authors.lastname: Muscal
authors.forename: Lorien
authors.initials: L
authors.lastname: Nassi
authors.forename: Andreas
authors.initials: A
authors.lastname: Reiff
authors.forename: Kenneth
authors.initials: K
authors.lastname: Schikler
authors.forename: Nora G
authors.initials: NG
authors.lastname: Singer
authors.forename: Dawn
authors.initials: D
authors.lastname: Wahezi
authors.forename: Amy
authors.initials: A
authors.lastname: Woodward
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D015267:Churg-Strauss Syndrome / Q000175:diagnosis; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005060:Europe; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis; D014890:Granulomatosis with Polyangiitis / Q000145:classification* / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D055953:Microscopic Polyangiitis / Q000175:diagnosis; D010865:Pilot Projects; D012015:Reference Standards; D012680:Sensitivity and Specificity; D012955:Societies, Medical; D014481:United States; D014657:Vasculitis / Q000175:diagnosis
chemical_list: D000305:Adrenal Cortex Hormones; D003520:Cyclophosphamide; D008727:Methotrexate