Table 1.

SSc criteria sets definition.

ACR 198011Leroy and Medsger 200112ACR/EULAR 201314
Major criteria: Scleroderma proximal to MCP/MTP joints
Minor criteria: Sclerodactyly, digital ulcers, bibasilar pulmonary fibrosis on chest radiograph
Presence of RP and (1) SSc-type nailfold capillary pattern and/or (2) SSc-related antibodies (ACA, anti Scl-70, anti-PM-Scl, anti-fibrillin, anti-RNA polymerase III, anti-Th/To)ItemsScore
Bilateral skin thickening proximal to the MCP joints9
Skin thickening of whole finger distal to MCP or puffy fingers4 or 2
Fingertip lesions: digital ulcers or pitting scars2 or 3
Telangiectasia2
Abnormal nailfold capillaries2
Pulmonary arterial hypertension or interstitial lung disease2
RP3
SSc-related antibodies (ACA, anti Scl-70, anti RNA polymerase III)3
Requires 1 major or 2 minor criteriaRequires either (1) or (2) if RP is objectively documented. Requires (1) and (2) if RP is subjectiveRequires a total score of 9 or more
  • ACR: American College of Rheumatology; EULAR: European League Against Rheumatism; SSc: systemic sclerosis; RP: Raynaud phenomenon; ACA: anticentromere antibodies; MCP: metacarpophalangeal; MTP: metatarsophalangeal.