特发性肺纤维化中肌成纤维细胞来源的研究进展

Research advance of myofibroblast origin in idiopathic pulmonary fibrosis

在特发性肺纤维化发病过程中，肌成纤维细胞是主要效应细胞，具有促进细胞外基质沉积、肺顺应性下降，肺组织结构破坏以及分泌多种炎性细胞因子的功能。因此，研究肌成纤维细胞的来源对于阐明特发性肺纤维化的发病机制以及寻求有效的分子治疗靶点具有非常重要的意义。迄今为止，肌成纤维细胞的来源集中在3个方面：肺内的成纤维细胞、肺组织上皮细胞和骨髓源性细胞或纤维细胞。本文对近年来的相关文献进行整理，加以阐述。

In the development of idiopathic pulmonary fibrosis（IPF）, myofibroblasts are the major effector cells, which can promote extracellular matrix deposition, decrease lung compliance, damage lung structure,and secrete many inflammatory cytokines in a variety of functions. Thus, the research for their origin is of great significance to clarify the pathogenesis of IPF and search for effective molecular therapeutic targets. To date, the myofibroblast sources are concentrated in lung fibroblasts,lung epithelial cells and bone marrow-derived cells or fibroblasts. This review mainly discusses the studies of the myofibroblast origin in IPF in recent years.