|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 fingers||2|
|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 ulcers||2|
|Fingertip pitting scars||3|
|Abnormal nailfold capillaries||–||2|
|Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2)||Pulmonary arterial hypertension||2|
|Interstitial lung disease||2|
|SSc-related autoantibodies (anticentromere, anti–topoisomerase I [anti–Scl-70], anti–RNA polymerase III) (maximum score is 3)||Anticentromere||3|
|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.