Therapeutic for treating age-associated lung fibrosis.
Idiopathic pulmonary fibrosis (IPF), the most common type of lung fibrosis, is a serious and deadly lung disease with no known cause and no known cure currently available. IPF is characterized by progressive scar tissue formation, causing irreversible damage to the lung, which can ultimately lead to respiratory failure. IPF has a median survival of less than 3 years, and patients typically present with established lung fibrosis at the time of diagnosis. IPF is widely regarded as a disease of aging. The incidence and prevalence of IPF increase with age; two-thirds of IPF patients are older than 60 years at the time of presentation, with a mean age of 66 years at the time of diagnosis. According to the American Lung Association, approximately 50,000 new cases are diagnosed each year. The US holds the largest IPF market with the highest number of diagnosed prevalent cases. Current FDA-approved therapies have been shown to slow the progression of lung decline, however, they fail to halt fibrosis progression and they do not reverse established fibrosis. Further, these therapies do not improve the quality of life for patients and there is only a modest survival benefit. There is a need to develop effective treatments which can promote the reversal of age-dependent established fibrosis.
Emory scientists have discovered that DMF treatment can inhibit the development pro-fibrotic myofibroblast phenotypes and it promotes the reversal established pro-fibrotic phenotypes in cells isolated from IPF patient lungs. They also found that lung-targeted (inhaled) delivery of DMF (but not oral systemic delivery) reduced lung oxidative stress levels and demonstrated efficacy for reversing age-dependent established fibrosis in an aging animal model of persistent lung fibrosis.
Inhaled delivery of DMF has proven to be effective for reducing lung oxidative stress and promoting the reversal of age-associated established lung fibrosis, whereas oral delivery failed to demonstrate efficacy.