Figure 2.

A proposed model to depict the pathogenesis of idiopathic pulmonary fibrosis caused by telomere dysfunction. Telomere dysfunction causes cellular senescence and/or cell death of alveolar epithelial type 2 cells (AEC2s), which leads to the production of a pro-fibrotic niche, mediated by the SASP. This probably leads to increased mechanical tension and a TGF-β signaling loop in a spatially distributed manner in the lung, owing to the inability to form new alveoli. Eventually, pulmonary fibrosis develops due to the significant increase in myofibroblasts.