Table 1

The American College of Rheumatology/European League Against Rheumatism criteria for the classification of systemic sclerosis*

ItemSub-item(s)Weight/score†
Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (sufficient criterion)9
Skin thickening of the fingers (only count the higher score)Puffy fingers2
Sclerodactyly of the fingers (distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints)4
Fingertip lesions (only count the higher score)Digital tip ulcers2
Fingertip pitting scars3
Telangiectasia2
Abnormal nailfold capillaries2
Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2)Pulmonary arterial hypertension2
Interstitial lung disease2
Raynaud's phenomenon3
SSc-related autoantibodies (anticentromere, anti–topoisomerase I [anti–Scl-70], anti–RNA polymerase III) (maximum score is 3)Anticentromere3
Anti–topoisomerase I
Anti–RNA polymerase III
  • *These criteria are applicable to any patient considered for inclusion in a systemic sclerosis study. The criteria are not applicable to patients with skin thickening sparing the fingers or to patients who have a scleroderma-like disorder that better explains their manifestations (eg, nephrogenic sclerosing fibrosis, generalised morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromyalgia, porphyria, lichen sclerosis, graft-versus-host disease, diabetic cheiroarthropathy).

  • †The total score is determined by adding the maximum weight (score) in each category. Patients witha total score of ≥9 are classified as having definite systemic sclerosis.

  • SSc, systemic sclerosis.