Scleroderma/Systemic Sclerosis (2013 EULAR/ACR Criteria)

Review EULAR/ACR criteria for scleroderma


The European League Against Rheumatism and the American College of Rheumatology (ACR/EULAR) classification criteria were published in 2013. It was determined that skin thickening of the fingers extending proximal to the metacarpophalangeal joints is sufficient for to classify a patient as systemic sclerosis.

In the absence of this finding, seven findings should be identified and score: 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.

Patients with a score ≥9 are considered to have definite systemic sclerosis (scleroderma). Sensitivity was 91% and specificity was 92% in the validation sample for the new classification criteria. This is far superior to the 75% and 72% for the 1980 ACR classification criteria.

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1. These criteria are not applicable to a) patients having a SSc-like disorder better explaining their manifestations (below) or b) patients with skin thickening sparing the fingers

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SSc-like disorders: nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromyalgia, porphyria, lichen sclerosis, graft versus host disease, and diabetic cheiropathy.

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