Progressive fibrosis of the skin and of various internal organs is the pathologic hallmark of systemic sclerosis (SSc). The fibrotic process results in disruption of the normal architecture of the affected organs and ultimately leads to their dysfunction and failure. Excessive collagen deposition in affected tissues is a central event in the pathogenesis of SSc and is responsible for most of its clinical manifestations. The extent and rate of progression of the fibrotic process determine the course and prognosis of SSc. Pharmacologic manipulation of the multiple steps involved in the complex pathway leading to exaggerated fibrogenesis offers a potentially successful approach to SSc treatment. An understanding of the intimate mechanisms responsible for abnormalities in the regulation of fibroblast function in SSc, therefore, may lead to the rational design of novel therapeutic interventions.
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