Idiopathic Pulmonary Fibrosis: A Review on Molecular and Cellular Mechanisms

Abstract

Idiopathic pulmonary fibrosis is a progressive disease characterized by extracellular matrix accumulation and altered mechanical properties of lung tissue. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is attracting considerable attention due to disease acceleration and substantial mortality. The biomechanical properties of tissues are sensed and responded to by mechanotransduction pathways that facilitate sensing of changes in mechanical cues by tissue resident cells and convert the mechanical signals into downstream biochemical signals. In this review pathways such as Wnt/β-catenin pathway, TGF-β/Smad signaling pathway and EMT in IPF, VEGF and its relation with PI3K-Akt signalling pathway, PI3-Akt pathway, PDGF Signalling Pathway, Hippo/YAP signalling, JAK/STAT pathway, Rnd3/ p190/Rho-Gap pathway have been discussed. This review also covers current therapeutic strategies in relation to idiopathic pulmonary fibrosis.