TY - JOUR
T1 - 2013 classification criteria for systemic sclerosis
T2 - An American college of rheumatology/European league against rheumatism collaborative initiative
AU - Van Den Hoogen, Frank
AU - Khanna, Dinesh
AU - Fransen, Jaap
AU - Johnson, Sindhu R.
AU - Baron, Murray
AU - Tyndall, Alan
AU - Matucci-Cerinic, Marco
AU - Naden, Raymond P.
AU - Medsger, Thomas A.
AU - Carreira, Patricia E.
AU - Riemekasten, Gabriela
AU - Clements, Philip J.
AU - Denton, Christopher P.
AU - Distler, Oliver
AU - Allanore, Yannick
AU - Furst, Daniel E.
AU - Gabrielli, Armando
AU - Mayes, Maureen D.
AU - Van Laar, Jacob M.
AU - Seibold, James R.
AU - Czirjak, Laszlo
AU - Steen, Virginia D.
AU - Inanc, Murat
AU - Kowal-Bielecka, Otylia
AU - Müller-Ladner, Ulf
AU - Valentini, Gabriele
AU - Veale, Douglas J.
AU - Vonk, Madelon C.
AU - Walker, Ulrich A.
AU - Chung, Lorinda
AU - Collier, David H.
AU - Csuka, Mary Ellen
AU - Fessler, Barri J.
AU - Guiducci, Serena
AU - Herrick, Ariane
AU - Hsu, Vivien M.
AU - Jimenez, Sergio
AU - Kahaleh, Bashar
AU - Merkel, Peter A.
AU - Sierakowski, Stanislav
AU - Silver, Richard M.
AU - Simms, Robert W.
AU - Varga, John
AU - Pope, Janet E.
PY - 2013/11
Y1 - 2013/11
N2 - 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.
AB - 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.
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U2 - 10.1136/annrheumdis-2013-204424
DO - 10.1136/annrheumdis-2013-204424
M3 - Article
C2 - 24092682
AN - SCOPUS:84885126599
SN - 0003-4967
VL - 72
SP - 1747
EP - 1755
JO - Annals of the rheumatic diseases
JF - Annals of the rheumatic diseases
IS - 11
ER -