2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative.
Document Type
Article
Publication Date
11-1-2013
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.
Recommended Citation
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 A.; Kahaleh, Bashar; Merkel, Peter A; Sierakowski, Stanislav; Silver, Richard M; Simms, Robert W; Varga, John; and Pope, Janet E, "2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative." (2013). Department of Medicine Faculty Papers. Paper 138.
https://jdc.jefferson.edu/medfp/138
PubMed ID
24122180
Comments
This article has been peer reviewed. It was published in: Arthritis and Rheumatism.
Volume 65, Issue 11, November 2013, Pages 2737-2747.
The published version is available at DOI: 10.1002/art.38098
Copyright © 2013 by the American College of Rheumatology