Research by Physical Therapy Professor Jim Mansoor and colleagues from UC Davis has contributed to a new drug treatment for the deadly lung disease idiopathic pulmonary fibrosis.
Physical Therapy professor’s research leads to new treatment of deadly lung diseaseResearch conducted by Dr. Jim Mansoor and colleagues from UC Davis has contributed to a new drug treatment for idiopathic pulmonary fibrosis, a deadly lung disease for which previously there has been no treatment outside of a lung transplant.
In 1997, Dr. Jim Mansoor, Professor - Department of Physical Therapy, and his colleagues from UC Davis launched a research project to study the effects of pirfenidone on a deadly lung disease, idiopathic pulmonary fibrosis (IPF), in animal models. The research was funded by Marnac, Inc who has since sold the rights to sell pirfenidone to InterMune. Today his preliminary work has contributed to a new treatment for patients who suffer from the disease.
Idiopathic pulmonary fibrosis occurs when the tissues in the lungs thicken and get stiffer which makes it hard for patients to breathe and intake oxygen. For the research, Dr. Mansoor and his team measured the effects of the drug by studying the forced vital capacity, the amount of air that is forcibly exhaled from the lungs. Dr. Mansoor's research found that animals who had induced IPF and treated with pirfenidone showed a significant improvement in forced vital capacity from animals that were not treated with pirfenidone. Pirfenidone helped reduce the scarring (thickening of tissues) in the lungs and improved pulmonary function in animals. The cause of IPF is often unknown and early screenings are not available. Currently, there are no successful treatments for the disease. The only other option is a lung transplant.
"When I was doing this research, I would receive emails from patients saying they've learned that there are no treatments for IPF but saw my research online and asked if I would recommend the drug. I would respond and apologize that I was not a medical doctor and that I could only say that the drug improved pulmonary function in animal models," commented Dr. Mansoor.
Patients who are diagnosed with the disorder are usually well into the disease and are usually middle-aged to older adults. Many patients die three to six years after diagnosis because of respiratory failure. Pirfenidone offers hope for patients. The drug was found to help slow down the deterioration of lung functions in recent human clinical trials potentially increasing life expectancy. Similar to Dr. Mansoor's animal model, the human clinical trials also showed a reduction in the deterioration in forced vital capacity.
"Many researchers do a lot of work not knowing if it will go anywhere. Learning that my work is going to have a direct impact on patients is rewarding," said Dr. Mansoor.
Currently, studies have found pirfenidone to be relatively safe, with no cardiovascular side effects but with the possibility of nausea and gastrointestinal (GI) distress. Although the effect of pirfenidone on IPF is a relatively new discovery, the drug itself has been around since the 1960s.
Pirfenidone is marketed in the United States under the name Esbriet® which is developed by InterMune. InterMune licensed certain rights to pirfenidone from Marnac, Inc. and its co-licensor, KDL GmbH, in 2002. In 2007 the company purchased from Marnac and KDL the rights to sell the compound under the patents in the US and several other countries.
Read more about Dr. Mansoor's study in a paper titled "Pirfenidone attenuates bleomycin-induced changes in pulmonary mechanics in hamsters" which was published in the Proceedings of the Society for Experimental Biology and Medicine journal in 1997.