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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 <16 years of age may be classified as having JSSc with the presence of one
major criterion and two minor criteria ( Table 101.5 ). The major criterion which is
required for every diagnosis is proximal sclerosis or induration of the skin. The minor
criteria include many clinical symptoms (e.g., sclerodactyly, RP, dysphagia,
arrhythmias), and several laboratory findings (e.g., positive ANA).
The presence of autoantibodies is supportive of the diagnosis. High titers of ANA
are reported in 80% to 97% of children with JSSc. While, antitopoisomerase I (antiScl-70) autoantibodies and anticentromere antibodies are occasionally positive, this is
seen less frequently in children than with adults. Routine blood tests (e.g., complete
blood cell count [CBC], serum chemistries, and urinalysis) are not helpful
diagnostically. In select cases, a skin biopsy may be required.