Table 2.
ACR/EULAR 2013 Systemic Scleroderma classification criteria. Adapted from Van Den Hoogen et al. [16].
| Items | 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 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 | 2 | |
| SSc-related autoantibodies (anticentromere, anti-topoisomerase I [anti–Scl-70], anti-RNA polymerase III) (maximum score is 3) | Anticentromere | 3 |
| Anti–topoisomerase I | 3 | |
| Anti–RNA polymerase III | 3 |
Abbreviations: SSc: systemic sclerosis. These criteria are applicable to any patient considered for inclusion in an SSc study. The criteria are not applicable to patients with skin thickenings paring the fingers or to patients who have a scleroderma-like disorder that better explains their manifestations (e.g., nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromyalgia, porphyria, lichen sclerosis, graft-versus-host disease, diabetic cheerio arthropathy). The total score is determined by adding the maximum weight (score) in each category. Patients with a total score of 9 are classified as having definite SSc.