Current American College of Rheumatology European League Against Rheumatism classification criteria for Systemic Sclerosis

What are the current American College of Rheumatology European League Against Rheumatism classification criteria for Systemic Sclerosis?

From Hoogen F, Khanna D, Fransen J, et al (2013). 2013 Classification criteria for systemic sclerosis. Arthritis Rheum 65: 2737-2747.

ItemsSub-itemsWeight
Skin thickening of fingers of both hands extending proximal to metacarpophalangeal (MCP) joints9
Skin thickening of fingers (only count the highest score)Puffy fingers
Whole finger, distal to MCP
2
4
Fingertip lesions (only count the highest score)Digital tip ulcers
Pitting scars
2
3
Telangiectasia2
Abnormal nailfold capillaries2
Pulmonary arterial hypertension and/or interstitial lung disease2
Raynaud’s phenomenon3
Scleroderma-related antibodies (any of anti-centromere, anti-topoisomerase I [anti-ScL 70], anti-RNA polymerase III)3
Patients with a total score of ≥9 are classified as having definite systemic sclerosis (sensitivity 91%, specificity 92%)

Other potential causes of skin thickening must be excluded prior to application of these criteria to a specific patient 

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