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A long time ago, in an airway far, far away: early changes in the proximal airways in IPF and their response to drug treatment

thoraxjnl · 2026-04-14 · canonical JSON source

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Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing lung disease characterised by irreversible remodelling of the distal lung and loss of respiratory function. Current models centre alveolar epithelial progenitor dysfunction in pathogenesis, with injured epithelial cells exhibiting deregulated stress responses and impaired differentiation, compromising effective regeneration.1 Recent single-cell studies highlight persistent transitional epithelial states and the emergence of aberrant basaloid cells, consistent with stalled or maladaptive repair.2 3 Epithelial dysfunction is closely linked to mesenchymal activation through dysregulated epithelial-mesenchymal cross-talk, promoting fibroblast expansion, excessive extracellular matrix deposition and progressive architectural distortion.4