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Author(s): Hanan Hosni, Fatma Taha, Hanan Darweesh, Heba Elwi and Mohamed El Basel
Scleroderma, or systemic sclerosis (SSc), is a multifactorial, chronic fibrotic collagen tissue disorder that is mainly based on 3 pathogenetic features: accumulation of extracellular matrix, vasculopathy of the small vessels, and autoimmunity. Clinical and experimental data show that the pathogenesis of SSc is multifactorial, involving both genetic and environmental Factors. The most prominent manifestations of SSc are abnormalities of the circulation and involvement of multiple organ systems, including the musculoskeletal, renal, pulmonary, cardiac, and gastrointestinal systems, with fibrotic and/or vascular Complications. Although cytokines and immune mediators play a major role in the initiation and progression of disease, the importance of the genetic component in SSc pathogenesis has not been fully defined.
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