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. 2013 Nov;65(11):2737-47.
doi: 10.1002/art.38098. Epub 2013 Oct 3.

2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative

Frank van den Hoogen  1 , Dinesh Khanna, Jaap Fransen, Sindhu R Johnson, Murray Baron, Alan Tyndall, Marco Matucci-Cerinic, Raymond P Naden, Thomas A Medsger Jr, 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 Seibold, Laszlo 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
Affiliations

2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative

Frank van den Hoogen et al. Arthritis Rheum. 2013 Nov.

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.

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References

    1. Wollheim FA. Classification of systemic sclerosis: visions and reality. Rheumatology (Oxford) 2005;44:1212–6. - PubMed
    1. Masi AT, Medsger TA, Jr, Rodnan GP, Fries JF, Altman RD, Brown BW, et al. Methods and preliminary results of the Scleroderma Criteria Cooperative Study of the American Rheumatism Association. Clin Rheum Dis. 1979;5:27–79.
    1. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee Preliminary criteria for the classification of systemic sclerosis (scleroderma) Arthritis Rheum. 1980;23:581–90. - PubMed
    1. Preliminary criteria for the classification of systemic sclerosis (scleroderma) Bull Rheum Dis. 1981;31:1–6. No authors listed. - PubMed
    1. Nadashkevich O, Davis P, Fritzler MJ. A proposal of criteria for the classification of systemic sclerosis. Med Sci Monit. 2004;10:CR615–21. - PubMed

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