ACR/EULAR classification criteria for systemic sclerosis
Entry criteria
Skin thickening involves the fingers
Alternative scleroderma-like disorder diagnoses causing skin thickening (such as nephrogenic sclerosing fibrosis, generalized morphea, eosinophilic fasciitis, scleredema diabeticorum, scleromyxedema, erythromelalgia, porphyria, lichen sclerosis, GvHD, diabetic cheiroarthropathy) are not present
Skin thickening
Skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints
Puffy fingers
Sclerodactyly of the fingers distal to the metacarpophalangeal joints but proximal to the proximal interphalangeal joints
None
Fingertip lesions
Digital tip ulcers
Fingertip pitting scars
None
Telangiectasia
Present
Absent
Abnormal nailfold capillaries
Present
Absent
Pulmonary arterial hypertension and/or ILD
Present
Absent
Raynaud's phenomenon
Present
Absent
Systemic sclerosis-related autoantibodies (anti-centromere, anti-Scl-70, and/or anti-RNA polymerase III)
Present
Absent
Please complete all sections.
Reference
Frank van den Hoogen, Dinesh Khanna, Jaap Fransen et al. 2013 classification criteria for systemic sclerosis: an American college of rheumatology / European league against rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55.
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