Background Systemic sclerosis (SS) is a connective tissue disease with inflammation, fibrosis and vascular damage. Early diagnosis may be difficult if disease expression is oligosymptomatic, presenting with only Raynaud's phenomenon or limited scleroderma (LS).
Methods The study group included 65 children and adolescents: 24 patients with SS and 41 patients with LS. Mean age was 12,7 years (SD±0,56) in patients with SS and 13 years (SD ±0,73) in patients with LS. Mean disease duration was 4,9 years (SD±0,81) in patients with SS and 5,1 years (SD ±0,42) in patients with LS.
Results Similar immunologic abnormalities in patients with SS and LS revealed. Pulmonary function testing documented restrictive ventilatory defect in 33,3% children and adolescents with SS and in 31,7% patients with LS. Alteration of gastrointestinal tract detected in 91,7% patients with SS and in 63,8% patients with LS. Inflammation of mucous of stomach and esophagus has mostly light degree of manifestation, low degree of activity and absence of Helicobacter pylori (60% patients with SS and 50% patients with LS). Repeatability of visceral changes as well as similar morphological character in skin lesion focuses in children and adolescents with SS and LS established.
Conclusions Evidence of common mechanisms of pathogenesis for systemic sclerosis and localized scleroderma is given.
Disclosure of Interest None declared
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