Updated Research On Pathogenesis And Pharmacological Treatment Of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis(IPF) is a chronic progressive interstitial pneumonia of unknown etiology, with complicated pathogenesis and limited treatment. The pathogenesis is summarized as follows: Genetic susceptibility and environment factors lead to that alveolar epithelial cells(AECs) are repeatedly injured and abnormally repaired, even lead to apoptosis. Damaged AECs release a variety of fibrosis factors represented by transforming growth factor-β, which induces a large number of fibroblasts and myofibroblasts are accumulated, activated and proliferated, and then the damaged AECs acquire the ability of resisting apoptosis. The fibroblasts and myofibroblasts secrete excessive extracellular matrix which is deposited in the lung, which starts the process of pulmonary fibrosis. With the aggravating of the pulmonary fibrosis, the pulmonary matrix is stiffer and stiffer, which promotes the pulmonary fibrosis aggravated by the positive feedback, leading to the irreversible progress of the disease. In recent years the studies confirm that there is not treatment effect by the traditional treatment n-acetylcysteine+prednisone+azathioprine, antioxidant n-acetylcysteine, anticoagulant warfarin, interferon gamma and endothelin receptor inhibitor bosentan and ambrisentan. Even there are security risks in these drugs. So they are not recommended in the application of clinical application. The latest clinical trials have shown that antifibrosis drug Pirfenidone and tyrosine kinase inhibitor Nintedanib can reduce the descent speed of lung function and put off the disease progression, bringing the glimmer of curing the disease. There are a variety of new drugs in early clinical trials, such as the monoclonal antibodies of interleukin-13, connective tissue growth factor, integrin αvβ6 and lysyl oxidase-like 2 and the antagonist of lysophosphatidic acid receptor 1. The result is worthy of expectation.