Abstract: Pulmonary fibrosis (PF) is a chronic progressive interstitial lung disease. The pathogenesis of PF is not yet clear. The two anti fibrosis drugs approved for IPF treatment, nidanib and pirfenidone, have been proved to reduce the decline of pulmonary function of PF, but both have side effects. So far, there is no obvious and effective treatment to prevent the progress of PF. Therefore, this review focuses on the different cells, molecular mechanisms involved in PF and the current treatment progress of PF, so as to provide theoretical support for a better understanding of these cells, molecular mechanisms and drug development and application in PF.

Key words: pulmonary fibrosis, epithelial cells, fibroblasts, epithelial-mesenchymal transition (EMT), macrophages, mitochondrion, telomere, treatment status