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 phenomenon | – | 3 |
SSc-related autoantibodies (anticentromere, anti-topoisomerase I [anti-Scl-70], anti-RNA polymerase III) (maximum score is 3) | Anticentromere Anti-topoisomerase I Anti-RNA polymerase III | 3 |
ACR: American College of Rheumatology; EULAR: European Alliance of Associations for Rheumatology (formerly the European League Against Rheumatism); RNA: ribonucleic acid; 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 thickening sparing the fingers or to patients who have a scleroderma-like disorder that better explains their manifestations (eg, nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromyalgia, porphyria, lichen sclerosus, graft-versus-host disease, diabetic cheiroarthropathy).
¶ 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.Do you want to add Medilib to your home screen?