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Systemic Sclerosis

Rare Diseases of the Immune System, ISSN: 2283-6403, Vol: Part F3423, Page: 105-126
2024
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Book Chapter Description

Systemic sclerosis (SSc) is a connective tissue disease characterized by fibrosis clinically evident in the skin (scleroderma), but which may also occur in other organs or systems. Fibrosis is the final stage of a pathogenetic process triggered by functional and structural alterations of the microcirculation encompassing platelet activation and dysregulation of the immune response. SSc affects the skin, blood vessels, musculoskeletal system, and internal organs (gastrointestinal tract, lungs, heart, kidneys). Visceral involvement is responsible for significant morbidity and mortality in SSc patients [1, 2].

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