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[DOI] [PMC free article] [PubMed] [Google Scholar] Chen L, Kwong M, Lu R, Ginzinger D, Lee C, Leung L, Chan JY

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The pathological core involves repeated micro-injury to alveolar epithelial cells, abnormal activation of fibroblasts, and excessive deposition of the extracellular matrix (ECM), ultimately leading to irreversible lung architecture destruction and progressive respiratory failure (Martinez et al., 2017