Idiopathic Pulmonary Fibrosis (IPF)

Idiopathic pulmonary fibrosis (IPF) is a progressive, usually fatal disease affecting approximately 130,000 patients in the United States, with nearly 50,000 new cases diagnosed annually. IPF has a mortality rate of nearly 40,000 each year – the same number of deaths as breast cancer. To date, no treatments or therapies have proven effective against IPF. Normal progression of the disease leads to death for 70 percent of patients within five years of diagnosis, and lung transplantation has been the only intervention.

Autoimmunity in Idiopathic Pulmonary Fibrosis (IPF)

Through their experiments, our researchers estimate that antigen-specific autoimmunity occurs in approximately 60 percent of IPF patients. This data suggests, for the first time, that IPF may not be “idiopathic,” rather, it may result due to autoimmunity.

Current immunologic treatments used to slow the progression of IPF are severely limited by the very nature of global immune suppression: non-specific suppression of the immune system and significant, treatment-limiting side effects. Conversely, the ideal treatment for autoimmune diseases would target only the implicated auto-antigen. The therapeutic strategy of immune tolerance uses the body’s normal immunologic processes to produce antigen-specific suppression of immune responses against the implicated auto-antigen believed to cause the disease.