Rachel Lynne Zemans, MD

Most patients survive only three years after being diagnosed with idiopathic pulmonary fibrosis (IPF). Current treatments somewhat slow the pace of deterioration but do not cure the disease or even improve lung function, shortness of breath, or survival. We know that low lung function and difficulty breathing in IPF are due to replacement of healthy lung tissue with scar, and that a cell called a fibroblast deposits this scar. In the healthy lung, fibroblasts do not make scar tissue and the cells responsible for absorbing oxygen, known as epithelial cells, are intact. Normally, if the epithelial cells are injured, such as by cigarette smoke or infection, they repair themselves. In IPF, the epithelial cells are unable to repair themselves, which activates the fibroblasts to deposit scar tissue. We aim to understand how the epithelial cells fail to repair and thus activate fibroblasts to make scar tissue. Our ultimate goal is to develop new therapies that will improve lung function, the ability to breathe, and survival.

Update:

In the first year of this grant, we have identified ways in which lung regeneration fails and promotes scar formation in IPF. These findings may ultimately translate into novel therapies to restore normal lung regeneration.