摘要 : Idiopathic pulmonary fibrosis (IPF)-a disease of exaggerated, relentless, and apparently irreversible scarring of lung parenchyma-remains a clinically challenging problem. Even with the recent approvals of pirfenidone and nintedan... 展开 Idiopathic pulmonary fibrosis (IPF)-a disease of exaggerated, relentless, and apparently irreversible scarring of lung parenchyma-remains a clinically challenging problem. Even with the recent approvals of pirfenidone and nintedanib for treatment of IPF, more efficacious drugs need to be developed. Rational drug development requires sufficient understanding of pathogenesis, but gaining such understanding has proven to be a challenge in IPF because of the manifest intricacy of the disease mechanisms. Molecular and cellular disturbances of several biologically fundamental pathways have been demonstrated, including epithelial injury and apoptosis, microvascular abnormalities, oxidative stress, coagulation disturbances, steroid-resistant inflammation, transdifferentiation of several precursor and mature cell types into activated myofibroblasts, and anomalous interactions of apoptosis-resistant myofibroblasts with the extracellular matrix. Although we have already learned quite a lot about the molecular details of each of these disease pathways in IPF, targeting all of them simultaneously seems an unachievable goal. There is, however, hope coming from the field of cytokine biology. 收起