2013 classification criteria for systemic sclerosis: An american college of rheumatology/European league against rheumatism collaborative initiative

Frank Van Den Hoogen, Dinesh Khanna, Jaap Fransen, Sindhu R. Johnson, Murray Baron, Alan Tyndall, Marco Matucci-Cerinic, Raymond P. Naden, Thomas A. Medsger, Patricia E. Carreira, Gabriela Riemekasten, Philip J. Clements, Christopher P. Denton, Oliver Distler, Yannick Allanore, Daniel E. Furst, Armando Gabrielli, Maureen D. Mayes, Jacob M. Van Laar, James R. SeiboldLaszlo Czirjak, Virginia D. Steen, Murat Inanc, Otylia Kowal-Bielecka, Ulf Müller-Ladner, Gabriele Valentini, Douglas J. Veale, Madelon C. Vonk, Ulrich A. Walker, Lorinda Chung, David H. Collier, Mary Ellen Csuka, Barri J. Fessler, Serena Guiducci, Ariane Herrick, Vivien M. Hsu, Sergio Jimenez, Bashar Kahaleh, Peter A. Merkel, Stanislav Sierakowski, Richard M. Silver, Robert W. Simms, John Varga, Janet E. Pope

Research output: Article

1139 Citations (Scopus)

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. Conclusion 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.

Original languageEnglish
Pages (from-to)2737-2747
Number of pages11
JournalArthritis and rheumatism
Volume65
Issue number11
DOIs
Publication statusPublished - nov. 1 2013

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ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Pharmacology (medical)

Cite this

Van Den Hoogen, F., Khanna, D., Fransen, J., Johnson, S. R., Baron, M., Tyndall, A., Matucci-Cerinic, M., Naden, R. P., Medsger, T. A., Carreira, P. E., Riemekasten, G., Clements, P. J., Denton, C. P., Distler, O., Allanore, Y., Furst, D. E., Gabrielli, A., Mayes, M. D., Van Laar, J. M., ... Pope, J. E. (2013). 2013 classification criteria for systemic sclerosis: An american college of rheumatology/European league against rheumatism collaborative initiative. Arthritis and rheumatism, 65(11), 2737-2747. https://doi.org/10.1002/art.38098