Novel Inhaled Therapy for Idiopathic Pulmonary Fibrosis

DESCRIPTION (provided by applicant): Idiopathic pulmonary fibrosis (IPF) is a deadly lung disease that affects an estimated 133,000 patients in the U.S. and more than 5 million worldwide. Median survival time after diagnosis is only 2-3 years, with a 5-year mortality rate exceeding 50%. IPF is the leading indication for lung transplant in most large treatment centers. The hallmark of the disease is progressive and ultimately fatal scarring in the lungs, detectable with high resolution computed tomography (HRCT) and lung biopsy. There is no consistent standard of care, and there are no FDA-approved agents to prevent, arrest, or reverse fibrosis. Consequently, a tremendous unmet need persists for the development of a truly disease-modifying drug for this indication. The goal of this phase I SBIR preclinical research program is to identify such an agent for IPF, which can then be further advanced for clinical evaluation in affected patients. The approach is based on increasing recognition of the role of a p