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Lookup NU author(s): Professor Jaap van Laar
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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.
Author(s): van Laar JM; van den Hoogen F; Khanna D; Fransen J; Johnson SR; Baron M; Tyndall A; Matucci-Cerinic M; Naden RP; Medsger TA; Carreira PE; Riemekasten G; Clements PJ; Denton CP; Distler O; Allanore Y; Furst DE; Gabrielli A; Mayes MD; Seibold JR; Czirjak L; Steen VD; Inanc M; Kowal-Bielecka O; Muller-Ladner U; Valentini G; Veale DJ; Vonk MC; Walker UA; Chung L; Collier DH; Csuka ME; Fessler BJ; Guiducci S; Herrick A; Hsu VM; Jimenez S; Kahaleh B; Merkel PA; Sierakowski S; Silver RM; Simms RW; Varga J; Pope JE
Publication type: Article
Publication status: Published
Journal: Annals of the Rheumatic Diseases
Year: 2013
Volume: 72
Issue: 11
Pages: 1747-1755
Print publication date: 01/11/2013
ISSN (print): 0003-4967
ISSN (electronic): 1468-2060
Publisher: BMJ Group
URL: http://dx.doi.org/10.1136/annrheumdis-2013-204424
DOI: 10.1136/annrheumdis-2013-204424
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