An Airway-Centric View of Idiopathic Pulmonary Fibrosis

Stancil et al offer an airway-centric view of idiopathic pulmonary fibrosis (IPF) which is the cumulative manifestation of countless spatially and temporally distinct microscopic foci of maladaptive repair in response to recurrent lung injury. However, the key drivers, essential cells, and critical mechanisms of maladaptive repair in IPF remain elusive. Decades of research has focused on alveolar injury and alveolar-mesenchymal cross-talk and their association with type II alveolar epithelial defects, fibroblast activation, and progressive lung fibrosis resulting in diminished organ function. In addition, it has been well documented that immune and endothelial cell types are invoked in IPF. However, the airway epithelium has been incompletely described in IPF even with significant evidence of airway-specific involvement.