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2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative

Open access
van den Hoogen, Frank, Khanna, Dinesh, Fransen, Jaap, Johnson, Sindhu R., Baron, Murray, Tyndall, Alan, Matucci‐Cerinic, Marco, Naden, Raymond P., Medsger, Thomas A., Carreira, Patricia E., Riemekasten, Gabriela, Clements, Philip J., Denton, Christopher P., Distler, Oliver, Allanore, Yannick, Furst, Daniel E., Gabrielli, Armando, Mayes, Maureen D., van Laar, Jacob M., Seibold, James R., Czirjak, Laszlo, Steen, Virginia D., Inanc, Murat, Kowal‐Bielecka, Otylia, Müller‐Ladner, Ulf, Valentini, Gabriele, Veale, Douglas J., Vonk, Madelon C., Walker, Ulrich A., Chung, Lorinda, Collier, David H., Csuka, Mary Ellen, Fessler, Barri J., Guiducci, Serena, Herrick, Ariane, Hsu, Vivien M., Jimenez, Sergio, Kahaleh, Bashar, Merkel, Peter A., Sierakowski, Stanislav, Silver, Richard M., Simms, Robert W., Varga, John, Pope, Janet E.·2013·Arthritis & Rheumatism

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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Metadata

Authors
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., Seibold, J. R., Czirjak, L., Steen, V. D., Inanc, M., Kowal‐Bielecka, O., Müller‐Ladner, U., Valentini, G., Veale, D. J., Vonk, M. C., Walker, U. A., Chung, L., Collier, D. H., Csuka, M. E., Fessler, B. J., Guiducci, S., Herrick, A., Hsu, V. M., Jimenez, S., Kahaleh, B., Merkel, P. A., Sierakowski, S., Silver, R. M., Simms, R. W., Varga, J., & Pope, J. E.
Venue
Arthritis & Rheumatism
Publisher
Wiley
Published
2013-10-03
Volume
65
Issue
11
Pages
2737-2747
ISSN
  • 0004-3591 (ISSN-L)
  • 1529-0131
OpenAlex
W2508060872
Cited by
3,409
References
22
FWCI
123.63
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: 2013 Classification Criteria for Systemic Sclerosis: An American College of Rheumatology/European League Against Rheumatism Collaborative Initiative. https://app.cochat.ai/papers/10.1002/art.38098
The paper
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 & Rheumatism. https://doi.org/10.1002/art.38098

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.