Idiopathic Pulmonary Fibrosis

Background

Idiopathic pulmonary fibrosis (IPF) is a disease characterized by progressive scarring of the lungs, which leads to dyspnea, cough, and ultimately respiratory failure. The median survival following diagnosis is 2.8-4.2 years, with only 20% reaching 5 years of survival. IPF is currently considered an Orphan Disease in the USA – meaning it affects fewer than 200,000 people. Drugs that the FDA grants orphan status qualify for a number of financial incentives and extended market exclusivity under the Orphan Drug Act of 1983.
Mechanistically, idiopathic pulmonary fibrosis is poorly understood. IPF is characterized as being primarily epithelial-driven and fibroblast-activated process, with a secondary inflammatory component as well. While normal lung injuries are repaired, IPF lungs lack this restorative capability.

There are currently 2 drugs approved for IPF in the United States, Esbriet® and Ofev®. Additional efforts to manage the disease include improving the quality-of-life of those afflicted. Lung transplantation is currently the only treatment available that has been shown to improve survival. However, lung transplantation is a risky procedure and the negative side-effects associated with it often outweigh any benefits.

Products in Development

New Amsterdam Sciences is currently developing and looking forward to moving into a Phase 1B/2A clinical trial with our lead compound, NAS150. NAS150 is a SOD mimetic, anti-oxidant and anti-inflammatory Mn-metalloporphyrin compound being developed for IPF. It has been shown to reduce, halt, and potentially reverse the progression of the disease in animal models, and has been well-tolerated in humans.

We are also developing NAS911, a novel, stem-cell active immunomodulatory compound that also modulates fibrogenesis. Studies to date have indicated success in the “gold standard” bleomycin model of IPF. NAS has explored the more clinically relevant model, radiation-induced pulmonary-fibrosis and observed efficacy in reducing the development of fibrosis. Using radiation as a model for IPF, allows us to use NAS150 as a treatment for radiation induced lung injury in side effects of cancer patients undergoing radiation therapy.

For additional information on the products being developed for this indication, please contact us.