Item | Sub-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 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 | |
Telangiectasia | – | 2 |
Abnormal nailfold capillaries | – | 2 |
Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2) | Pulmonary arterial hypertension | 2 |
Interstitial lung disease | 2 | |
Raynaud's phenomenon | – | 3 |
SSc-related autoantibodies (anticentromere, anti–topoisomerase I [anti–Scl-70], anti–RNA polymerase III) (maximum score is 3) | Anticentromere | 3 |
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.