title: Assessing the performance of the Birmingham Vasculitis Activity Score at diagnosis for children with antineutrophil cytoplasmic antibody-associated vasculitis in A Registry for Childhood Vasculitis (ARChiVe).
abstract: OBJECTIVE
  There are no validated tools for measuring disease activity in pediatric vasculitis. The Birmingham Vasculitis Activity Score (BVAS) is a valid disease activity tool in adult vasculitis. Version 3 (BVAS v.3) correlates well with physician's global assessment (PGA), treatment decision, and C-reactive protein in adults. The utility of BVAS v.3 in pediatric vasculitis is not known. We assessed the association of BVAS v.3 scores with PGA, treatment decision, and erythrocyte sedimentation rate (ESR) at diagnosis in pediatric antineutrophil cytoplasmic antibody-associated vasculitis (AAV).
  
  
  METHODS
  Children with AAV diagnosed between 2004 and 2010 at all ARChiVe centers were eligible. BVAS v.3 scores were calculated with a standardized online tool (www.vasculitis.org). Spearman's rank correlation coefficient (r(s)) was used to test the strength of association between BVAS v.3 and PGA, treatment decision, and ESR.
  
  
  RESULTS
  A total of 152 patients were included. The physician diagnosis of these patients was predominantly granulomatosis with polyangiitis (n = 99). The median BVAS v.3 score was 18.0 (range 0-40). The BVAS v.3 correlations were r(s) = 0.379 (95% CI 0.233 to 0.509) with PGA, r(s) = 0.521 (95% CI 0.393 to 0.629) with treatment decision, and r(s) = 0.403 (95% CI 0.253 to 0.533) with ESR.
  
  
  CONCLUSIONS
  Applied to children with AAV, BVAS v.3 had a weak correlation with PGA and moderate correlation with both ESR and treatment decision. Prospective evaluation of BVAS v.3 and/or pediatric-specific modifications to BVAS v.3 may be required before it can be formalized as a disease activity assessment tool in pediatric AAV.
authors.affiliation: British Columbia Children’s Hospital, Vancouver, British Columbia, Canada.
authors.forename: Kimberly
authors.initials: K
authors.lastname: Morishita
authors.forename: Suzanne C
authors.initials: SC
authors.lastname: Li
authors.forename: Eyal
authors.initials: E
authors.lastname: Muscal
authors.forename: Steven
authors.initials: S
authors.lastname: Spalding
authors.forename: Jaime
authors.initials: J
authors.lastname: Guzman
authors.forename: America
authors.initials: A
authors.lastname: Uribe
authors.forename: Leslie
authors.initials: L
authors.lastname: Abramson
authors.forename: Kevin
authors.initials: K
authors.lastname: Baszis
authors.forename: Susanne
authors.initials: S
authors.lastname: Benseler
authors.forename: Suzanne
authors.initials: S
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
authors.initials: G
authors.lastname: Higgins
authors.forename: Anne
authors.initials: A
authors.lastname: Eberhard
authors.forename: Kaleo
authors.initials: K
authors.lastname: Ede
authors.forename: Lisa
authors.initials: L
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: Marisa
authors.initials: M
authors.lastname: Klein-Gitelman
authors.forename: Erica F
authors.initials: EF
authors.lastname: Lawson
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: Kabita
authors.initials: K
authors.lastname: Nanda
authors.forename: Lorien
authors.initials: L
authors.lastname: Nassi
authors.forename: Kathleen M
authors.initials: KM
authors.lastname: O'Neil
authors.forename: Egla
authors.initials: E
authors.lastname: Rabinovich
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
authors.initials: K
authors.lastname: Schikler
authors.forename: Anne
authors.initials: A
authors.lastname: Stevens
authors.forename: Dawn
authors.initials: D
authors.lastname: Wahezi
authors.forename: David A
authors.initials: DA
authors.lastname: Cabral
mesh_terms: D000328:Adult; D000367:Age Factors; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D001799:Blood Sedimentation; D002097:C-Reactive Protein / Q000378:metabolism; D002648:Child; D015331:Cohort Studies; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D006801:Humans; D008297:Male; D010372:Pediatrics / Q000592:standards; D012042:Registries / Q000592:standards*; D015203:Reproducibility of Results; D012189:Retrospective Studies; D012219:Rheumatology / Q000592:standards; D012720:Severity of Illness Index; D014657:Vasculitis / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology*
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D002097:C-Reactive Protein