European Respiratory Journal 2023 61: 2300407; DOI: 10.1183/13993003.00407-2023

Extract

Pulmonary fibrosis, including idiopathic pulmonary fibrosis (IPF), is a debilitating and often fatal lung disorder characterised by the abnormal accumulation of extracellular matrix (ECM), leading to fibrotic scarring and lung stiffening [1]. Although the exact mechanism is yet to be fully understood, aberrant tissue remodelling with excess ECM is a critical factor in the progression of pulmonary fibrosis [1, 2]. Myofibroblasts are specialised fibroblasts that express α-smooth muscle actin (α-SMA) and can exert contractile forces, in addition to their involvement in production of pathological ECM components, including fibrillar collagens I and III, and fibronectin [3]. This excessive fibrotic ECM deposition results in increased stiffness, which further activates transforming growth factor (TGF)-β from a latent form and stimulates myofibroblast differentiation and production of profibrotic mediators, such as connective tissue growth factor, thus perpetuating a vicious cycle of fibrosis [3, 4].

Focusing on EndMT, Wu et al. discovered that EC-like myofibroblasts are able to differentiate into myofibroblasts, resulting in a significant contribution to the pathogenesis of pulmonary fibrosis, and this process is redirected through induction of FoxA2 https://bit.ly/3YQvSIX

Footnotes

• Conflict of interest: T. Yanagihara reports grants from Boehringer Ingelheim, outside the submitted work. C. Guignabert reports grants from Acceleron Pharma (Cambridge, MA, USA), a wholly owned subsidiary of Merck & Co., Inc. (Rahway, NJ, USA), Corteria pharmaceutical, and Structure therapeutics (ex ShouTi), outside of submitted work. M.R.J. Kolb reports being site PI for sponsored clinical trials for Roche and Boehringer Ingelheim, grants from Canadian Institute for Health Research, grants and personal fees for advisory board work from Boehringer Ingelheim and Roche Canada, a chief editor allowance from the European Respiratory Society, personal fees for advisory board work from Horizon, Algernon, CSL Behring, DevPro, Bellerophon and BMS, personal fees for adjudication committee work from United Therapeutics, and personal fees for data monitoring committee work from LabCorp, outside the submitted work.

• Received March 8, 2023.
• Accepted March 11, 2023.

• Copyright ©The authors 2023. For reproduction rights and permissions contact permissions@ersnet.org

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