Grant: Project Grant

Idiopathic pulmonary fibrosis (IPF) is characterized by increasing lung stiffness that eventually makes breathing impossible. This pernicious disease has a mortality rate of 40% within 3-5 years of diagnosis and is characterized by a substantial reduction in quality of life. IPF is incurable and few medications exist to combat this disease. Therefore, delineating novel therapeutic targets is pressing to combat this significant unmet medical need. While the precise cause(s) of IPF is unknown, the pathology is promoted by unrestrained activation of fibroblasts, which deposit the extracellular matrix (ECM) that causes tissue stiffness and is also associated with macrophages. The proposed research program aims to i) define how fibroblasts polarize and activate macrophages in IPF, ii) determine how macrophages activate fibroblasts to drive IPF pathogenesis and iii) delineate the development and trajectory of pathogenic fibroblasts in IPF. This work will facilitate the development of innovative and translational strategies to abate IPF and provide foundational insights into the molecular mechanisms that control activation of fibroblasts, the ECM producing cells in IPF. Overall, the research will leverage cutting-edge laboratory approaches to increase health outcomes for those diagnosed with IPF and other fibrotic diseases.