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
Performance:
• The sensitivity is 90%, specificity 96%.
