The Pediatric Rheumatology European Society (PRES), American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) developed criteria for the diagnosis of systemic sclerosis in pediatric patients.


Major criteria:

(1) presence of proximal induration (sclerosis) of the skin


Minor criteria:

(1) sclerodactyly

(2) Raynaud's phenomenon

(3) capillary abnormalities in the nailfold

(4) ulcers at the digital tips

(5) dysphagia

(6) gastroesophageal reflux

(7) renal failure

(8) new onset of arterial hypertension

(9) cardiac arrhythmias

(10) heart failure

(11) pulmonary fibrosis

(12) diffusion capacity of carbon monoxide (DLCO) < 80% of predicted

(13) pulmonary hypertension

(14) friction rubs over tendons

(15) arthritis

(16) myositis

(17) peripheral neuropathy

(18) carpal tunnel syndrome

(19) antinuclear antibodies

(20) presence of autoantibodies associated with systemic sclerosis: anticentromere, antitopoisomerase I, antifibrillarin, antifibrillin, anti-PM-Scl, anti-RNA polymerase I, anti-RNA polymerase III


Diagnosis of juvenile systemic sclerosis requires:

(1) age < 16 years

(2) major criteria present

(3) >= 2 minor criteria



• The sensitivity is 90%, specificity 96%.


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