In these cases, treatment may consist of immune-suppressing drugs, anti-inflammatories, and/or removing the exposure.
However, often there’s no known cause of interstitial lung disease. In this case, the disease falls into a class called idiopathic interstitial pneumonias, the most common of which is idiopathic pulmonary fibrosis (IPF). “It’s a disease where people get scarring in their lungs without inflammation preceding it,” say Lake Morrison, MD, assistant professor of medicine (Pulmonary, Allergy and Critical Care Medicine). “It’s an unusual situation: scarring begets scarring. It’s a devastating disease that not a lot of people know about.”
The first thing Duke offers is an accurate diagnosis, which may have been overlooked for some time because the initial symptoms — shortness of breath and coughing — are often attributed to aging or to cardiovascular disease. Although it can occur in a wide variety of people, Morrison says it occurs mostly in people over 50, with a typical patient being a 70-year-old white male with a history of smoking.
“If it’s IPF, the pathway is referral for lung transplant if they are a candidate, and trying to manage symptoms, pulmonary rehab, oxygen,” Morrison says.
Although there are currently no medicines approved for IPF in the United States, a drug already approved in other countries may become available here sometime in 2014 or 2015 after the results of a recent clinical trial have been fully analyzed and reported. “IPF progresses inexorably and insidiously over time. If we ever get a medicine that slows things down, that’s a big improvement,” Morrison says.
Patients at Duke can have access to potential therapies by participating in clinical trials. Morrison says there have been more than 15 clinical trials at Duke in the seven years he’s been here.
The drug likely to be approved this year or next is an anti-fibrotic, but Morrison says how it works is not fully understood. “The drugs that will work are novel — they haven’t been invented or are just being tested now,” he says. “Lots of thing show promise in a petri dish and when you try to put it in humans, it doesn’t work.”
If lung transplant become necessary, Duke patients have access to one of the best lung transplant teams in the country.