Grant: Operating Grant

Idiopathic pulmonary fibrosis is a chronic degenerative disease of the lungs for which there exists limited effective treatment. Approximately half of the affected patients will die within 3 years of diagnosis. The disease mostly affects individuals who are 50 years and older, and is more common in certain families as well as in ex-smokers. Recent research has revealed that patients with this disease accumulate within their air sacs large amounts of a protein that is normally found in mucus. This protein is called mucin. We believe that by blocking access of lung cells to oxygen, mucin chokes the air sacs, causing damage, scarring and the accumulation of even more mucin. The excessive amount of mucus in the air sacs (alveoli) activates genes and proteins seen only when tissue oxygen levels are low (hypoxia). We have observed that certain products derived from plants and existing drugs reverse the adverse effects of these hypoxia-driven genes, and prevent some of the disease mechanisms. We will therefore explore new molecules that can slow down the damage, and stop the scarring of the air sacs in patients with IPF. In order to quantify the clinical effect of new therapies for IPF, we will also study the anti-fibrotic capacity of these compounds in a mouse model. Finally, we will conduct a small clinical trial in patients in which similar compounds are delivered through the diet. We hope to better understand how the scarring occurs in IPF and what new treatments are most effective.