title: 2013 American College of Rheumatology/European League against rheumatism classification criteria for systemic sclerosis outperform the 1980 criteria: data from the Canadian Scleroderma Research Group.
abstract: OBJECTIVE
  The goal of this study was to determine the sensitivity of the new 2013 classification criteria for systemic sclerosis (SSc; scleroderma) in an independent cohort of SSc subjects and to assess the contribution of individual items of the criteria to the overall sensitivity.
  
  
  METHODS
  SSc subjects from the Canadian Scleroderma Research Group cohort were assessed. Sensitivity was determined in several subgroups of patients. In patients without the criterion of skin thickening proximal to the metacarpophalangeal (MCP) joints, we recalculated sensitivity after removing the individual criterion.
  
  
  RESULTS
  A total of 724 SSc patients were included. Most were women (86%), mean age was 55.8 years, mean disease duration was 10.9 years, and 59% had limited cutaneous SSc (lcSSc). Overall, the sensitivity of the 2013 criteria was 98.3% compared to 88.3% for the 1980 criteria. This pattern was consistent among those with lcSSc (98.8% versus 85.6%), anticentromere antibodies (98.9% versus 79.8%), disease duration ≤3 years (98.7% versus 84.7%), and no skin involvement proximal to the MCP joints (97% versus 60%). In the latter subgroup, removing Raynaud's phenomenon and sclerodactyly from the criteria reduced the sensitivity to 77% and 79%, respectively. Removing both sclerodactyly and puffy fingers reduced the sensitivity to 62%.
  
  
  CONCLUSIONS
  The 2013 SSc classification criteria classify more SSc patients than the 1980 criteria. The improvement in sensitivity is most striking in those with lcSSc, especially those without skin involvement proximal to the MCP joints. The addition of Raynaud's phenomenon and puffy fingers to the 2013 criteria accounts for important gains in sensitivity.
authors.affiliation: McGill University and Jewish General Hospital, Montreal, Quebec, Canada.
authors.forename: Hebah
authors.initials: H
authors.lastname: Alhajeri
authors.forename: Marie
authors.initials: M
authors.lastname: Hudson
authors.forename: Marvin
authors.initials: M
authors.lastname: Fritzler
authors.forename: Janet
authors.initials: J
authors.lastname: Pope
authors.forename: Solène
authors.initials: S
authors.lastname: Tatibouet
authors.forename: Janet
authors.initials: J
authors.lastname: Markland
authors.forename: David
authors.initials: D
authors.lastname: Robinson
authors.forename: Niall
authors.initials: N
authors.lastname: Jones
authors.forename: Nader
authors.initials: N
authors.lastname: Khalidi
authors.forename: Peter
authors.initials: P
authors.lastname: Docherty
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Kaminska
authors.forename: Ariel
authors.initials: A
authors.lastname: Masetto
authors.forename: Evelyn
authors.initials: E
authors.lastname: Sutton
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Mathieu
authors.forename: Sophie
authors.initials: S
authors.lastname: Ligier
authors.forename: Tamara
authors.initials: T
authors.lastname: Grodzicky
authors.forename: Sharon
authors.initials: S
authors.lastname: LeClercq
authors.forename: Carter
authors.initials: C
authors.lastname: Thorne
authors.forename: Geneviève
authors.initials: G
authors.lastname: Gyger
authors.forename: Douglas
authors.initials: D
authors.lastname: Smith
authors.forename: Paul R
authors.initials: PR
authors.lastname: Fortin
authors.forename: Maggie
authors.initials: M
authors.lastname: Larché
authors.forename: Murray
authors.initials: M
authors.lastname: Baron
mesh_terms: D000328:Adult; D000368:Aged; D002170:Canada / Q000453:epidemiology; D015331:Cohort Studies; D003430:Cross-Sectional Studies; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012216:Rheumatic Diseases / Q000145:classification* / Q000175:diagnosis / Q000453:epidemiology; D012219:Rheumatology / Q000145:classification* / Q000592:standards; D012594:Scleroderma, Localized / Q000145:classification* / Q000175:diagnosis / Q000453:epidemiology; D012595:Scleroderma, Systemic / Q000145:classification* / Q000175:diagnosis / Q000453:epidemiology; D012955:Societies, Medical / Q000592:standards*; D014481:United States / Q000453:epidemiology; D055815:Young Adult