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Innate immune signaling and stem cell renewal in idiopathic pulmonary fibrosis

Nature Medicine volume 22, pages 1210–1212 (2016)Cite this article

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Aberrant injury responses in the distal lung likely underpin the development of idiopathic pulmonary fibrosis (IPF). A recent study shows that defective Toll-like receptor 4 (TLR4)-mediated hyaluronan binding impairs alveolar type 2 cell renewal, which may contribute to a dysregulated lung-injury response in IPF.

IPF is the most common and severe interstitial lung disease, with a 5-year survival rate of around 20–30%—a rate that is on par with many aggressive forms of cancer1. Alarmingly, although the incidence of IPF is increasing worldwide, there are currently no curative therapeutic options to halt disease progression. This in part reflects our lack of understanding of the pathogenic initiators and drivers that underlie this condition. Similarly to most fibrotic conditions, the fibrotic response probably reflects an abnormal wound-healing program maintained by dysregulated epithelial–mesenchymal cross-talk. Mechanistically, most attention has been focused on the myofibroblast as the key effector cell of the fibrogenic response. Indeed, the only approved therapies for IPF, nintedanib and pirfenidone, probably affect fibroblast proliferation and extracellular matrix (ECM) production1.

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Figure 1: AEC2 cell renewal and response to injury is impaired in IPF.

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Authors and Affiliations

  1. UCL Division of Medicine, Paul F. Mercer and Rachel C. Chambers are in the Centre for Inflammation and Tissue Repair, UCL Respiratory, University College London, London, United Kingdom.,

    Paul F Mercer & Rachel C Chambers

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  1. Paul F Mercer
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  2. Rachel C Chambers
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Correspondence to Rachel C Chambers.

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Mercer, P., Chambers, R. Innate immune signaling and stem cell renewal in idiopathic pulmonary fibrosis. Nat Med 22, 1210–1212 (2016). https://doi.org/10.1038/nm.4230

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