Vascular Contribution to Lung Repair and Fibrosis

Lungs are constantly exposed to environmental perturbations and therefore have remarkable capacity to regenerate in response to injury. Sustained lung injuries, aging, and increased genomic instability, however, make lungs particularly susceptible to disrepair and fibrosis. Pulmonary fibrosis constitutes a major cause of morbidity and is often relentlessly progressive, leading to death from respiratory failure. The pulmonary vasculature, which is critical for gas exchanges and plays a key role during lung development, repair, and regeneration, becomes aberrantly remodeled in patients with progressive pulmonary fibrosis. Although capillary rarefaction and increased vascular permeability are recognized as distinctive features of fibrotic lungs, the role of vasculature dysfunction in the pathogenesis of pulmonary fibrosis has only recently emerged as an important contributor to the progression of this disease. This review summarizes current findings related to lung vascular repair and regeneration and provides recent insights into the vascular abnormalities associated with the development of persistent lung fibrosis.

Automated summary

Lungs have the ability to regenerate in response to injury, but sustained injuries, aging, and genomic instability make them susceptible to disrepair and fibrosis. Pulmonary fibrosis is a major cause of morbidity, and the dysfunction of the pulmonary vasculature contributes to its progression. This review summarizes current findings related to lung vascular repair and provides insights into the development of persistent lung fibrosis.