Grant: Operating Grant

Idiopathic pulmonary fibrosis (IPF) is one of the most severe chronic lung diseases. It affects approximately 8,000-13,000 Canadians, half of them dying within 3-4 years of diagnosis. The last decade has shown how complex the underlying biology of IPF is. Much of the basic research has focused on studying the initiators of the disease, and thus it is not surprising that most of the currently developed drugs target these earlier steps of the disease. There is now a first drug approved to treat IPF, but it does not affect the high mortality. The research proposed in this grant will investigate the mechanisms of progression rather than initiation of the disease, assuming that they are more critical at the time when the diagnosis is established and patients are seen by physicians. Recent evidence has shown that the abnormal scar tissue is not only the end product in IPF, but plays a very active role in progressive build up of scars by modifying lung cells and triggering them to behave abnormally. The active role of accumulating scar tissue in IPF will be the central piece of this research proposal.