Agostini Carlo, Gurrieri Carmela
Department of Clinical and Experimental Medicine, Clinical Immunology and Hematology Branches, Padua University School of Medicine, Padua, Italy.
Proc Am Thorac Soc. 2006 Jun;3(4):357-63. doi: 10.1513/pats.200601-010TK.
Idiopathic pulmonary fibrosis (IPF) is a progressive and irreversible fibrosing lung disease of unknown etiology and with an unfavorable outcome, leading ultimately to death due to respiratory failure. To date, no treatment strategies have been effective in modifying the natural course of IPF and its fatal outcome. The aberrant parenchymal remodeling is characterized by the expansion of fibroblasts/myofibroblasts, which form characteristic subepithelial foci, and by the abnormal deposition of extracellular matrix in the lung parenchyma. Although the pathophysiology underlying this disease has not yet been fully elucidated, animal models of pulmonary fibrosis have provided contributions in dissecting the molecular basis of this disease, focusing on the role of cytokines and chemokines as important pathogenetic mediators of lung fibrosis. Starting with the data obtained from animal models, this article provides a comment on a number of findings that suggest the possible role of the chemokine/cytokine system in the pathogenesis of IPF.
特发性肺纤维化（IPF）是一种病因不明、预后不良的进行性、不可逆性纤维化肺病，最终可导致呼吸衰竭死亡。迄今为止，尚无治疗策略能有效改变IPF的自然病程及其致命结局。异常的实质重塑表现为成纤维细胞/肌成纤维细胞的扩增，形成特征性的上皮下病灶，以及肺实质中细胞外基质的异常沉积。尽管该疾病的病理生理学尚未完全阐明，但肺纤维化动物模型为剖析该疾病的分子基础做出了贡献，重点关注细胞因子和趋化因子作为肺纤维化重要发病介质的作用。本文从动物模型获得的数据出发，对一些表明趋化因子/细胞因子系统在IPF发病机制中可能作用的研究结果进行评论。
