First atlas of cells involved in lung fibrosis could lead to new treatments

3D rendering of a lung

Researchers have created an atlas of all the cells involved in the chronic lung disease idiopathic pulmonary fibrosis (IPF), a breakthrough that could improve treatment of the condition and related lung diseases, according to a NHLBI-funded study published in Science Advances.

IPF is a chronic disease that causes the tissue surrounding the air sacs, or alveoli, in the lungs to becomes thick and stiff for unknown reasons. Over time, this can result in permanent scarring or fibrosis in the lungs that make it progressively more difficult to breathe.

The research team from Yale University and Baylor College of Medicine created the atlas by analyzing 330,000 cells in 79 human lungs. They used a technique called single-cell RNA sequencing that allowed them to detail changes in each cell.

The atlas is already helping reveal how IPF affects lung cells. The researchers found that cells in the lungs that are responsible for respiration make a misguided attempt at self-repair that instead gives rise to a different type of cell that specialize in air delivery. The research also led to the discovery of a new type of call involved in scarring called aberrant basaloid cells.

The researchers say that these changes might help explain why people with IPF develop breathing difficulties. They suggest that in addition to scarring, lung fibrosis is also caused by an abnormal repair process. The researchers say if they are able to better understand the faulty repair, they may be able to develop treatments that could ameliorate it.

A separate group of researchers from the Vanderbilt University Medical Center and the Translational Genomics Research Institute (TGen) published similar findings in the same issue of Science Advances. They found previously unknown genetic and cellular changes in the lungs of people with pulmonary fibrosis.  Using single-cell RNA sequencing in more than 100,000 lung cells from 20 pulmonary fibrosis patients, the researchers identified several types of cells that had not previously been detected. The findings could help lead to new ways to prevent or treat the lung condition.