Fig. 1: Overview of mechanisms contributing to the pathophysiology of IPF.
From: Emerging roles of noncoding RNAs in idiopathic pulmonary fibrosis

Repeated injury to AECs leads to the dysregulation of their self-renewal and repair processes, accompanied by the activation of macrophages, which results in the aberrant secretion of cytokines and fibrogenic growth factors. This secretion recruits and activates fibroblasts and promotes their differentiation into myofibroblasts. These myofibroblasts produce excessive ECM and alter mechanical stiffness, triggering fibrosis and the remodeling of pulmonary architecture. (Created with BioRender.com).