2013 ACR/EULAR Systemic Sclerosis Classification Criteria

Applies the 2013 ACR/EULAR weighted criteria across skin, vascular, lung, and autoantibody findings to classify systemic sclerosis, for research/cohort definition, not a diagnostic test, and not applicable when finger skin is spared or an SSc-like disorder better explains the findings.

Exclusion gate: finger skin spared OR an SSc-like disorder (e.g. nephrogenic systemic fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, GVHD) better explains the findings

Skin thickening of the fingers of BOTH hands extending proximal to the MCP joints (sufficient criterion)

Skin thickening of the fingers (count the higher score only)

Fingertip lesions (count the higher score only)

Telangiectasia

Abnormal nailfold capillaries

Pulmonary arterial hypertension (PAH)

Interstitial lung disease (ILD)

PAH and ILD form a single “PAH and/or ILD” item capped at 2 points; having both still contributes only 2.

Raynaud's phenomenon

Anti-centromere antibody

Anti-topoisomerase I (anti-Scl-70) antibody

Anti-RNA polymerase III antibody

The three SSc-related autoantibodies form a single category capped at 3 points; any one, two, or all three positive still contributes only 3.