title: 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.
abstract: OBJECTIVE
  The 1980 American College of Rheumatology (ACR) classification criteria for systemic sclerosis (SSc) lack sensitivity for early SSc and limited cutaneous SSc. The present work, by a joint committee of the ACR and the European League Against Rheumatism (EULAR), was undertaken for the purpose of developing new classification criteria for SSc.
  
  
  METHODS
  Using consensus methods, 23 candidate items were arranged in a multicriteria additive point system with a threshold to classify cases as SSc. The classification system was reduced by clustering items and simplifying weights. The system was tested by 1) determining specificity and sensitivity in SSc cases and controls with scleroderma-like disorders, and 2) validating against the combined view of a group of experts on a set of cases with or without SSc.
  
  
  RESULTS
  It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for the patient to be classified as having SSc; if that is not present, 7 additive items apply, with varying weights for each: skin thickening of the fingers, fingertip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary arterial hypertension, Raynaud's phenomenon, and SSc-related autoantibodies. Sensitivity and specificity in the validation sample were, respectively, 0.91 and 0.92 for the new classification criteria and 0.75 and 0.72 for the 1980 ACR classification criteria. All selected cases were classified in accordance with consensus-based expert opinion. All cases classified as SSc according to the 1980 ACR criteria were classified as SSc with the new criteria, and several additional cases were now considered to be SSc.
  
  
  CONCLUSIONS
  The ACR/EULAR classification criteria for SSc performed better than the 1980 ACR criteria for SSc and should allow for more patients to be classified correctly as having the disease.
authors.affiliation: St. Maartenskliniek and Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
authors.forename: Frank
authors.initials: F
authors.lastname: van den Hoogen
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.forename: Jaap
authors.initials: J
authors.lastname: Fransen
authors.forename: Sindhu R
authors.initials: SR
authors.lastname: Johnson
authors.forename: Murray
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authors.lastname: Baron
authors.forename: Alan
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authors.lastname: Tyndall
authors.forename: Marco
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authors.lastname: Matucci-Cerinic
authors.forename: Raymond P
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authors.forename: Thomas A
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authors.lastname: Medsger
authors.forename: Patricia E
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authors.lastname: Carreira
authors.forename: Gabriela
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authors.lastname: Riemekasten
authors.forename: Philip J
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authors.lastname: Clements
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Denton
authors.forename: Oliver
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authors.lastname: Distler
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
authors.forename: Armando
authors.initials: A
authors.lastname: Gabrielli
authors.forename: Maureen D
authors.initials: MD
authors.lastname: Mayes
authors.forename: Jacob M
authors.initials: JM
authors.lastname: van Laar
authors.forename: James R
authors.initials: JR
authors.lastname: Seibold
authors.forename: Laszlo
authors.initials: L
authors.lastname: Czirjak
authors.forename: Virginia D
authors.initials: VD
authors.lastname: Steen
authors.forename: Murat
authors.initials: M
authors.lastname: Inanc
authors.forename: Otylia
authors.initials: O
authors.lastname: Kowal-Bielecka
authors.forename: Ulf
authors.initials: U
authors.lastname: Müller-Ladner
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
authors.forename: Douglas J
authors.initials: DJ
authors.lastname: Veale
authors.forename: Madelon C
authors.initials: MC
authors.lastname: Vonk
authors.forename: Ulrich A
authors.initials: UA
authors.lastname: Walker
authors.forename: Lorinda
authors.initials: L
authors.lastname: Chung
authors.forename: David H
authors.initials: DH
authors.lastname: Collier
authors.forename: Mary Ellen
authors.initials: ME
authors.lastname: Csuka
authors.forename: Barri J
authors.initials: BJ
authors.lastname: Fessler
authors.forename: Serena
authors.initials: S
authors.lastname: Guiducci
authors.forename: Ariane
authors.initials: A
authors.lastname: Herrick
authors.forename: Vivien M
authors.initials: VM
authors.lastname: Hsu
authors.forename: Sergio
authors.initials: S
authors.lastname: Jimenez
authors.forename: Bashar
authors.initials: B
authors.lastname: Kahaleh
authors.forename: Peter A
authors.initials: PA
authors.lastname: Merkel
authors.forename: Stanislav
authors.initials: S
authors.lastname: Sierakowski
authors.forename: Richard M
authors.initials: RM
authors.lastname: Silver
authors.forename: Robert W
authors.initials: RW
authors.lastname: Simms
authors.forename: John
authors.initials: J
authors.lastname: Varga
authors.forename: Janet E
authors.initials: JE
authors.lastname: Pope
mesh_terms: D032921:Consensus; D003953:Diagnosis-Related Groups; D006801:Humans; D012219:Rheumatology; D012595:Scleroderma, Systemic / Q000145:classification* / Q000175:diagnosis* / Q000276:immunology; D012680:Sensitivity and Specificity