Eric LeFebvre, MD, Chief Medical Officer at Pliant Therapeutics, describes the mechanism of action of PLN-74809, an orphan drug in development for treating patients with idiopathic pulmonary fibrosis (IPF).

IPF is a chronic, progressive, fibrosing lung disease with few treatment options and a poor prognosis. Common symptoms of IPF include shortness of breath and difficulty performing daily activities, such as walking and talking. Currently, there is no pharmacological cure for IPF with neither of the approved two therapies demonstrating an ability to stop the progression of the disease. 

As Dr. LeFebvre explains, PLN-74809 is a small-molecule, dual selective inhibitor of αVβ1 / αVβ6 integrin for IPF. These integrins cause upstream activation of TGF-β in actively fibrotic tissue, leading to increased collagen production and, ultimately, fibrosis. Inhibition of these integrins will block TGF-β activation, thereby preventing the growth of fibrotic tissue within the lung and bile ducts.

To learn more about IPF and other rare lung disease, visit checkrare.com/diseases/lung-diseases/