ID on Idiopathic Fibrosis
Jun 08, 2022 Cedars-Sinai Staff
Mechanisms behind idiopathic pulmonary fibrosis (IPF), a dangerous disease that scars the lungs and impairs breathing, are being demystified by Cedars-Sinai investigators. The condition affects more than 100,000 people in the U.S., with most patients dying within five years of diagnosis unless they receive a transplant.
In normal lungs, type 2 alveolar epithelial cells (ATII) function as progenitor or stem cells, regenerating to support lung renewal and health. Meanwhile, other stem cells support their ATII kin by secreting growth factors. But in IPF, these mechanisms are lost, leading to progressive scarring that disrupts the flow of oxygen into the bloodstream.
To understand why, investigators created a mouse model of pulmonary fibrosis in which they eliminated the growth hormone receptor. The results showed that mice without these receptors were more prone to developing fibrosis.
These findings suggest that treatment with growth hormone receptors using a nanosize delivery mechanism may improve lung function.