Dr. Frankel noted that, in addition to the above completed randomized trials, major studies are either ongoing or planned for other novel potential therapies for interstitial lung disease. Among the agents under scrutiny are etanercept (Enbrel), inhaled iloprost (Ventavis), imatinib mesylate (Gleevec), antitransforming growth factor-b, and anticonnective tissue growth factor.
In addition, National Jewish will participate in a phase III U.S. clinical trial of pirfenidone this spring. Pirfenidone, owned by InterMune, is a novel antifibrotic agent, until now studied mostly outside the United States.
In response to an audience question, Dr. Frankel said that although he has no experience with imatinib in IPF, investigators participating in the ongoing clinical trial tell him they think they're seeing a dramatic effect. The data haven't been unblinded yet, but results should be available in about a year.
But even if imatinib and the other investigational therapies ultimately prove ineffective, the fact remains that contemporary therapy for interstitial lung disease is more promising than most physicians think.
“It's common to see a lot of nihilism about interstitial lung diseases such that patients are assumed to have a very poor prognosis and not respond to therapy. However, that's not necessarily true anymore. Entities such as hypersensitivity pneumonitis and primary connective tissue diseases are eminently treatable,” Dr. Frankel said.