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Ann Rheum Dis 72:1747-1755 doi:10.1136/annrheumdis-2013-204424
  • Criteria

2013 classification criteria for systemic sclerosis: an American college of rheumatology/European league against rheumatism collaborative initiative

Table 1

The American College of Rheumatology/European League Against Rheumatism criteria for the classification of systemic sclerosis*

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.

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