2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative

F Hoogen, D Khanna, J Fransen, SR Johnson, M Baron… - 2013 - deepblue.lib.umich.edu
F Hoogen, D Khanna, J Fransen, SR Johnson, M Baron, A Tyndall, M Matucci‐Cerinic…
2013deepblue.lib.umich.edu
Objective. The 1980 American College of Rheuma-tology (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 …
Objective. The 1980 American College of Rheuma-tology (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 consensusbased expert opinion. All cases classified as SSc accord-
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