Idiopathic pulmonary fibrosis (IPF) is a progressive form of interstitial lung disease. In IPF, lung tissue becomes scarred, resulting in breathing difficulties. While the lung damage caused by pulmonary fibrosis is irreparable, palliative therapies can ease symptoms and improve quality of life.
Restoration of the alveolar epithelium is a desirable therapeutic endpoint of IPF. In the injured lungs, production of inflammatory signaling molecules recruit stem/progenitor cells to the site of injury to restore the integrity of the epithelial layer. Stem cells can differentiate into lung epithelial and endothelial cells, ameliorating lung injury and fibrosis. Both structural engraftment and/or paracrine/immunomodulatory effects produced by stem cell therapy may be responsible for therapeutic benefits in IPF.