Role of Growth Factors in the Pathogenesis of Systemic-Sclerosis-Associated Fibrosis

Systemic Sclerosis (SSc) is a systemic autoimmune disease of unknown etiology characterized by a severe fibroproliferative vasculopathy and frequently progressive cutaneous and internal organ fibrosis. The small-vessel vasculopathy and the tissue fibrotic alterations are responsible for the most serious clinical and pathological manifestations of the disease and for its high mortality. Despite the high severity and frequent mortality, there are currently no optimal therapeutic approaches for SSc, and its complex pathogenesis has not been fully elucidated. Numerous studies have suggested that growth factors and related regulatory macromolecules released from inflammatory and other cells present in the affected tissues play a crucial role in the frequently progressive cutaneous and visceral fibrosis. Here, we will review some of the recent studies describing the role of various growth factors and related macromolecules in the development and progression of the fibrotic process in SSc.