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

• Published in: Annals of the rheumatic diseases Vol. 72; no. 11; pp. 1747 - 1755
• Main Authors: 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, Ellen Csuka, Mary, 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
• Format: Journal Article Conference Proceeding
• Language: English
• Published: England BMJ Publishing Group Ltd and European League Against Rheumatism 01.11.2013; BMJ Publishing Group LTD
• Subjects: Access to information; Adult; Aged; Autoantibodies - blood; Classification; Clinical medicine; Committees; Decision analysis; Decision making; Disease Activity; Europe; Experts; Familial Primary Pulmonary Hypertension; Female; Humans; Hypertension, Pulmonary - etiology; Lung Diseases, Interstitial - etiology; Male; Middle Aged; Raynaud disease; Raynaud Disease - etiology; Reproducibility of Results; Researchers; Rheumatic diseases; Rheumatology; Scleroderma; Scleroderma, Limited - classification; Scleroderma, Limited - complications; Scleroderma, Limited - diagnosis; Scleroderma, Systemic - classification; Scleroderma, Systemic - complications; Scleroderma, Systemic - diagnosis; Sensitivity and Specificity; Studies; Systemic Sclerosis; Telangiectasis - etiology; Treatment; United States; United States; Europe; Systemic Sclerosis; Disease Activity; Treatment
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2013-204424

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, seven 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.