ABSTRACT

Scleroderma (systemic sclerosis; SSc) is a chronic autoimmune/ inflammatory disease leading to by widespread fibrosis of the skin and internal organs. The pathogenesis of SSc is not fully known due to its rarity and clinical heterogeneity. However, it is assumed that its pathogenesis is characterized by the triad of vasculopathy, immune activation and fibrosis, nowadays. Our understanding about the pathogenesis is considerably increased by recent studies. However, etiologic factors triggering the pathogenic process and genetic background are not fully determined. Although it is known how fibroblasts, the main actors of fibrogenesis are activated, it is not adequately understood how the fibroblastic activity is persistent. The automacity and resistance to apoptosis of fibroblasts and the transition of non-fibroblastic cells-to-fibroblastic cells are responsible for the persistence of fibroblastic activity. It is demonstrated that neovascularisation (angiogenesis and vasculogenesis) supplying inflammatory cells and proteolytic enzymes required for restoration of fibrotic tissue is failured in SSc. The cause of deficient neovascularisation remains a mystery.