局限性硬皮病其病因、发病机制复杂,与转化生长因子(TGF-β)及其受体有一定关系。TGF-β是细胞外基质(ECM)合成与沉积失调导致组织纤维化最重要的调节因子。研究认为TGF-β与靶细胞的细胞膜上TGF-β受体结合,激活Smads信号通路,上调细胞外基质的基因转录和翻译。现就TGF-β与局限性硬皮病发病关系的研究进展做一综述。 Limitations Scleroderma its etiology, pathogenesis is complex, and transforming growth factor (TGF-β) and its receptor have a certain relationship. TGF-β is the most important regulator of tissue fibrosis due to the imbalance between extracellular matrix (ECM) synthesis and deposition. It is believed that TGF-β binds to the TGF-β receptor on the cell membrane of target cells, activating the Smads signaling pathway and up-regulating gene transcription and translation of extracellular matrix. The progress on the relationship between TGF-β and localized scleroderma is reviewed.