Progress in signaling pathways related to the fibrosis mechanism in systemic sclerosis / 中华微生物学和免疫学杂志

ABSTRACT

Systemic sclerosis (SSc) is a complex and chronic autoimmune disease causing fibrosis in multiple tissues and organs. The cause of fibrosis may be related to the production of extracellular matrix (ECM) such as type Ⅰ and Ⅲ collagens, elastin and fibronectin resulting from the transformation of activated fibroblasts (FB) into muscle-forming fibroblasts (MFB). ECM could seriously damage the skin, lung, heart, kidney and other organs. At present, many studies have shown that several signaling pathways including TGF-β, mTOR, JAK/STAT, Wnt/β-Catenin, Hedgehog and Notch are involved in the proliferation of FB and the transformation of FB to MFB, which led to the production and deposition of a great quantity of ECM. Therefore, this article reviewed the relationship between these signaling pathways and fibrosis in SSc, hoping to provided reference for the development of effective treatments for SSc.