Chilosi M, Doglioni C, Murer B, Poletti V
Department of Pathology, University of Verona, Policlinico G. B. Rossi, 37134 Verona, Italy.
Sarcoidosis Vasc Diffuse Lung Dis. 2010 Jul;27(1):7-18.
New paradigms have been recently proposed in the pathogenesis of idiopathic pulmonary fibrosis (IPF), evidencing that in IPF the cumulative action of an accelerated parenchymal senescence determined by either telomere dysfunction or genetic defects, together with the concurrent noxious activity of tobacco smoking, are able to severely compromise the regenerative potential of parenchymal epithelial stem cells, triggering a cascade of molecular signals and events (scarring, bronchiolar proliferation, abnormal remodelling) eventually leading to severe and irreversible functional impairment. New pathogenic schemes focus on the complex molecular mechanisms driving in a vicious circle the different signalling pathways (e.g. Wnt/ -catenin, TGF-beta, caveolin-1, etc.) potentially involved in epithelial-mesenchymal transition and irreversible lung remodelling.
最近在特发性肺纤维化（IPF）的发病机制方面提出了新的范式，表明在IPF中，由端粒功能障碍或基因缺陷所决定的加速实质细胞衰老的累积作用，与吸烟同时存在的有害作用一起，能够严重损害实质上皮干细胞的再生潜力，引发一系列分子信号和事件（瘢痕形成、细支气管增殖、异常重塑），最终导致严重且不可逆的功能损害。新的致病机制集中在驱动不同信号通路（如Wnt/β-连环蛋白、转化生长因子-β、小窝蛋白-1等）陷入恶性循环的复杂分子机制上，这些信号通路可能参与上皮-间质转化和不可逆的肺重塑。
