Approximately 50,000 new cases of idiopathic pulmonary fibrosis (IPF) are diagnosed in the United States every year. In most cases the disease progresses rapidly, leading to death within 3 to 5 years. It has long been believed that chronic inflammation of the lungs was the cause of IPF—which is characterized by scarring of the lung tissue—but researchers at Massachusetts General Hospital (MGH) have put forth a new theory.
These investigators analyzed fluid from the lung surface of a mouse model of IPF and hypothesized that the activity of lysoposphatidic acid (LPA) acting through its receptor, LPA1, is responsible for attracting fibroblasts known to be present in affected lung tissue in IPF.
Lead investigator, Andrew Tager, MD, of the MGH pulmonary and critical care unit, said that identifying the key role of this pathway will give researchers a new therapeutic strategy. He added, “An agent that blocks this pathway is already being developed as a potential cancer treatment, and we’re hoping to be able to test it in our animal model of IPF to determine whether it might be a candidate for trials in patients.”
Study results will appear in the January 2008 issue of Nature Medicine and have received early online release.
