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Serious illness and death can occur in JSSc Severe, uncontrolled hypertension and rapidly progressive renal failure (scleroderma renal crisis) have been major causes of mortality, although the introduction of ACE inhibitors has dramatically improved shortterm survival Primary myocardial disease with conduction disturbances, pericarditis, and intractable CHF, as well as pulmonary hypertension caused by fibrosis, remains significant sources of morbidity and mortality Additional complications of JSSc include (i) digital gangrene and nonhealing ulcers most frequently involving the fingers, elbows, and malleoli secondary to vascular occlusion; (ii) disordered motility of the distal esophagus with dysphagia and reflux esophagitis (60% of affected children); (iii) malabsorption syndrome; (iv) thrombocytopenia with subsequent cerebral hemorrhage; (v) interstitial lung disease; and (vi) cranial nerve involvement with trigeminal sensory neuropathy, facial weakness, and tinnitus In 2007, a provisional classification system for scleroderma was established by the Pediatric Rheumatology European Society (PRES), the American College of Rheumatology (ACR), and the European League Against Rheumatism (EULAR) Patients