Pulmonary fibrosis is characterised by excessive scarring in the lung which leads to compromised lung function, serious breathing problems and in some diseases, death. It includes several lung disorders with idiopathic pulmonary fibrosis (IPF) the most common and most severe. Pulmonary fibrosis is considered to be perpetuated by aberrant wound healing which leads to fibroblast accumulation, differentiation and activation, and deposition of excessive amounts of extracellular matrix (ECM) components, in particular, collagen. Recent studies have identified the importance of changes in the composition and structure of lung ECM during the development of pulmonary fibrosis and the interaction between ECM and lung cells. There is strong evidence that increased matrix stiffness induces changes in cell function including proliferation, migration, differentiation and activation. Understanding how changes in the ECM microenvironment influence cell behaviour during fibrogenesis, and the mechanisms regulating these changes, will provide insight for developing new treatments.

肺纤维化的特征是肺部过度瘢痕化，导致肺功能受损，严重的呼吸问题以及某些疾病甚至死亡。它包括几种最常见，最严重的伴有特发性肺纤维化（IPF）的肺部疾病。肺纤维化被认为由于伤口异常愈合而永久存在，这导致成纤维细胞积累，分化和激活，以及沉积过多的细胞外基质（ECM）成分，特别是胶原蛋白。最近的研究已经确定了在肺纤维化发展以及ECM与肺细胞之间相互作用期间改变肺ECM的组成和结构的重要性。有充分的证据表明，增加的基质刚度会诱导细胞功能发生变化，包括增殖，迁移，分化和激活。了解ECM微环境的变化如何在纤维发生过程中影响细胞行为，以及调节这些变化的机制，将为开发新的治疗方法提供见识。