Latest progress on the molecular mechanisms of idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a serious life-threatening lung disease, and the median survival period of PF patients after diagnosis is only 2.5-3.5 years. At present, there are no effective drugs or therapeutics to reverse or even inhibit IPF. The main pathological characteristics of pulmonary fibrosis (PF) include damage to alveolar epithelial cells, fibroblast activation and extracellular matrix accumulation, which gradually lead to damage to the lung structure and decreased lung function. It is important to understand the cellular and molecular mechanisms of PF comprehensively and clearly. In this paper, critical signaling pathways related to PF were reviewed to present updates on the molecular mechanisms of PF.

Keywords: Alveolar epithelial cells; Idiopathic pulmonary fibrosis; Lung fibroblasts; Mitochondria; Wnt.