U. enlists patients in clinical study linked to fatal lung disease

A University of Utah physician is enrolling patients in a clinical study to test a potential medication for a rare and always-fatal lung disease.

There's no effective treatment or cure for pulmonary fibrosis, which affects more than 200,000 patients nationally. And experts caution that the number is probably actually much higher because of misdiagnosis.

It's a disease that's characterized by scarring of the lung caused by inflammation of the air sacs and tissues. Over time, the air sacs are replaced by fibrotic tissue, and eventually the lung can no longer transfer oxygen into the bloodstream. Each year, about 40,000 patients die — roughly the same number as die of breast cancer, according to the Pulmonary Fibrosis Foundation.

Dr. Mary Beth Scholand, a lung specialist at the U. School of Medicine, is enrolling patients with idiopathic pulmonary fibrosis for the four-year study of Interferon gamma-1b, a naturally occurring substance in the body's immune system. Already used to treat some cancers, the drug is now being tested for effectiveness in preventing scarring of the lungs caused by the disease.

The pulmonary fibrosis typically strikes people in their mid-60s or older, but it can occur in patients at much younger ages because the early symptoms are "insidious," Scholand said. They include shortness of breath, dry cough and sometimes crackling in the lungs that can be heard through a stethoscope. Those may be accompanied by fatigue and weakness, chest discomfort, loss of appetite and rapid weight loss.

Often, people don't recognize their own symptoms or that they could be a disease process, chalking the breathlessness or that small cough up to signs that they're aging or out of shape, Scholand said.

Before a diagnosis is made, various tests are run, including a CT scan, pulmonary function test, physical examination and, if indicated, a biopsy. Because the disease is usually diagnosed in an advanced stage, the average life expectancy now is three to five years after diagnosis. That's a picture that could change drastically with early detection, should suitable treatment be found.

"It's uniformly fatal," Scholand said, "but we can't predict the rate it will progress. If we could diagnose people earlier, we could see if interventions help."

It is the first clinical trial in Utah involving the disease, she said. Up to this point, patients have had to travel to Denver to get into the study.

To participate, one must have idiopathic pulmonary fibrosis, be 40 to 79 and willing to take three shots a week for two years — either the Interferon or a placebo.