The 2013 ACR/EULAR Criteria for the classification of Systemic Sclerosis
| 1. These criteria are applicable to any patient considered for inclusion in a SSc study. | ||
| 2. These criteria are not applicable to: | ||
| a) Patients having a SSc-like disorder better explaining their manifestations, such as: nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromyalgia, porphyria, lichen sclerosis, graft versus host disease, and diabetic cheiropathy. b) Patients with `Skin thickening sparing the fingers‘, | ||
| Items | Sub-items | 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 highest score) | Puffy fingers Sclerodactyly of the fingers (distal to MCP but proximal to the PIPs) | 2 4 |
| Finger tip lesions^ (only count the highest score) | Digital Tip Ulcers Finger Tip Pitting Scars | 2 3 |
| Telangiectasia | 2 | |
| Abnormal nailfold capillaries | 2 | |
| Pulmonary arterial hypertension and/or Interstitial lung Disease* (*Maximum score is 2) | PAH ILD | 2 |
| Raynaud’s phenomenon | 3 | |
| Scleroderma related antibodies** (any of anti-centromere, anti-topoisomerasel | Anti-centromere Anti-topoisomerasel | 3 |
| [anti-Sd 70], anti-RNA polymerase III) (**Maximum score is 3) | Anti-RNA polymerase III | |
| TOTAL SCORE^: | ||
Patients having a total score of 9 or more are being classified as having definite systemic sclerosis.
^Add the maximum weight (score) in each category to calculate the total score.
PAH is pulmonary arterial hypertension. The definition is proven PAH by right heart catheterization. ILD is interstitial lung disease defined as pulmonary fibrosis on HRCT or chest radiograph, most pronounced in the basilar portions of the lungs, or presence of `velcro’ crackles on auscultation not due to another cause such as congestive heart failure.
Rheumatology TT 