Robert Guzy, M.D., Ph.D.

Idiopathic pulmonary fibrosis (IPF) causes progressive scarring of the lungs, severe breathlessness, and too little oxygen. The cause of IPF is unknown. Despite current FDA-approved antifibrotic therapies, overall survival is just 3-5 years after diagnosis. Substances called Fibroblast Growth Factors (FGFs) have been implicated in pulmonary fibrosis, but their exact contribution is unclear. Our initial research suggests that FGF2 administration may decrease pulmonary fibrosis severity. We plan to define the mechanism by which FGF2 decreases pulmonary fibrosis, and determine its potential for therapeutic use.

Update: We have found that treating mice with FGF2 significantly decreases pulmonary fibrosis, and decreases the number of activated fibroblasts (the principal active cells of connective tissue) in response to bleomycin treatment. This suggests that FGF2 may be of therapeutic benefit in pulmonary fibrosis. During the coming year we will explore the antifibrotic mechanisms of FGF2 in lung fibroblasts in animal and cell culture models. We will also determine whether alterations in FGF2 signaling are present in lung fibroblasts isolated from patients with IPF.