Hope for a Devastating Disease

While search for a cure progresses, U-M scientists hope to slow damage

Idiopathic pulmonary fibrosis, or IPF, is a disease that affects hundreds of thousands of Americans, and kills 40,000 of them every year. In a process scientists don’t understand, the disease gradually destroys air sacs in the lung and replaces them with scar tissue, making it difficult and eventually impossible for patients to breathe. In most cases, the cause of IPF remains a mystery.

At the University of Michigan, clinicians and scientists are working together to unravel the mysteries surrounding IPF. They hope their research will lead to therapeutic drugs to block the scar-forming process or diagnostic tests that could make early detection possible.

“IPF is very hard to diagnose, and the diagnosis is often missed for months, and sometimes years, before it is recognized,” says Kevin R. Flaherty, M.D., an assistant professor of internal medicine in the Medical School, noting that early diagnosis is important in the management of IPF.

Most people believe IPF is the result of injury to the lung, followed by an abnormal healing process, Flaherty says. Exposure to toxic environmental agents like beryllium and silica dust can trigger IPF, and smoking is probably a related factor in many cases. Genetics almost certainly plays a role, as the disease tends to run in families.

Once diagnosed, there are no effective treatments. Flaherty says lung transplantation is an option for some patients, but many have other diseases that make a transplant impossible. And there simply aren’t enough donated lungs for everyone who needs one.

Bethany B. Moore, Ph.D., an assistant professor of internal medicine, and Galen B. Toews, M.D., professor of internal medicine and chief of pulmonary and critical care medicine, have identified biochemical signals that attract cells called fibrocytes to damaged lung tissue — one of the first steps in a chain of events leading to the formation of scar tissue in the lungs.

“We may not be able to stop the initial disease process, but perhaps we could keep it from progressing so rapidly,” Moore adds. “It’s a first step, but an important one, in solving the mystery of this disease. Right now, research is the only hope we can offer IPF patients.”

—Katie Gazella and Sally Pobojewski

For an expanded version of the story:
www.med.umich.edu/opm/newspage/2006/fibrosis.htm

For patient information on IPF:
www.med.umich.edu/intmed/pulmonary/patients/fibrosis.htm