NHLBI IN THE PRESS

Protein linked to lung cancer found to drive idiopathic pulmonary fibrosis in mice

Research has shown that cancer cells produce PD-L1 to evade attack from the immune system. Now there is evidence that a similar mechanism may occur in idiopathic pulmonary fibrosis (IPF), a chronic, progressive lung disease that causes buildup of scar tissue in the lungs and prevents oxygen transport to the bloodstream.

By examining lung tissue from patients with IPF, researchers found that invasive fibroblasts, a type of cell in normal conditions that helps to make connective tissue, produce high levels of PD-L1. These abnormal cells hide from the body’s immune system. To validate this finding, researchers then used mice to show that the severity of the disease could be reduced by inhibiting PD-L1.

The findings suggest that currently available anti-cancer drugs could be a therapeutic strategy to treat IPF. The study, partly funded by NHLBI, appeared in JCI Insight, a publication of the American Society for Clinical Investigation.