Irreversibility of Pulmonary Fibrosis.

Qing Yang Yu,Xiao Xiao Tang

doi:10.14336/ad.2021.0730

Abstract

Pulmonary fibrosis, a kind of terminal pathological changes in the lung, is caused by aberrant wound healing, deposition of extracellular matrix (ECM), and eventually replacement of lung parenchyma by ECM. Pulmonary fibrosis induced by acute lung injury and some diseases is reversible under treatment. While idiopathic pulmonary fibrosis is persistent and irreversible even after treatment. Currently, the pathogenesis of irreversible pulmonary fibrosis is not fully elucidated. The known factors associated with the development of irreversible fibrosis include apoptosis resistance of (myo)fibroblasts, dysfunction of pulmonary vessel, cell mitochondria and autophagy, aberrant epithelia hyperplasia and lipid metabolism disorder. In this review, other than a brief introduction of reversible pulmonary fibrosis, we focus on the underlying pathogenesis of irreversible pulmonary fibrosis from the above aspects as well as preclinical disease models, and also suggest directions for future studies.

Highlights

Pulmonary fibrosis, a kind of terminal pathological changes in the lung, is caused by aberrant wound healing, deposition of extracellular matrix (ECM), and eventually replacement of lung parenchyma by ECM
The apoptosis resistance of lung fibroblasts as well as dysfunction of mitochondrion, pulmonary vessel and lipid metabolism contribute to development of persistent pulmonary fibrosis
Aberrant epithelial hyperplasia and cell autophagy potentially cause the irreversibility of pulmonary fibrosis

Summary

Introduction

A kind of terminal pathological changes in the lung, is caused by aberrant wound healing, deposition of extracellular matrix (ECM), and eventually replacement of lung parenchyma by ECM. Idiopathic pulmonary fibrosis (IPF) is the most common fibrotic lung disease with progressive and irreversible development [3, 4]. Fas signaling dysfunction caused by down-regulation of Fas or BLM model of Fas overexpression of anti-apoptotic protein induces lung fibroblasts resistant deficiency genetic mice to apoptosis and retain the pro-fibrotic phenotype.

Results

Conclusion