Journal
AGING AND DISEASE
Volume 13, Issue 1, Pages 73-86Publisher
INT SOC AGING & DISEASE
DOI: 10.14336/AD.2021.0730
Keywords
pulmonary fibrosis; irreversibility; pathogenesis; lung
Categories
Funding
- National High-Level Talents Program
- National Natural Science Foundation of China [81770015]
- Local Innovative and Research Teams Project of Guangdong Pearl River Talents Program [2017BT01S155]
- Open Project of State Key Laboratory of Respiratory Disease [SKLRD-OP202109]
- Special Fund for Science and Technology Innovation of Guangdong Province [2020B1111330001]
- Guangzhou Institute of Respiratory Health Open Project (China Evergrande Group)Project [2020GIRHHMS16]
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Pulmonary fibrosis is a complex end-stage pathological change in the lung, with reversible fibrosis typically caused by acute lung injury or certain diseases, while idiopathic pulmonary fibrosis remains persistent and irreversible even after treatment. The pathogenesis of irreversible fibrosis, which involves factors such as apoptosis resistance, dysfunction of pulmonary vessels, mitochondria, and autophagy, as well as aberrant epithelial hyperplasia and lipid metabolism disorder, is not fully understood.
: 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 somediseases isreversible 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 irreversiblefibrosis 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 onthe underlying pathogenesis of irreversible pulmonary fibrosis from the above aspects as well aspreclinical disease models, and also suggestdirections for future studies
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