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Dr. Neil Ettinger, St. Luke's Hospital

Idiopathic pulmonary fibrosis in women on the rise

You may have never heard of idiopathic pulmonary fibrosis, or IPF, but about 40,000 people in the U.S. die from it each year. And while more men are diagnosed with it, IPF in women is on the rise.

IPF is a disease that results in fibrosis, or scarring, of the lungs. The scarring that happens in people with IPF is harmful because it thickens the air sacs of the lungs and causes a loss of the tissue's ability to transport oxygen, which makes breathing difficult.

The primary symptoms of IPF include shortness of breath and a dry cough. When physicians examine the lungs of people with IPF using a stethoscope, they usually hear a crackling sound in the bottom part of the lungs. A team approach to diagnosis, involving radiologists, pathologists and pulmonologists, is utilized in order to ensure a patient is not suffering from another condition.

Unfortunately, IPF is often fatal within a few years of diagnosis. There is no known cause for IPF, hence the term "idiopathic," which means "of unknown cause."

Scientists think IPF may be the result of the body's overreaction to healing what would otherwise seem to be an ordinary injury to the lung. Damage to lung tissue from a respiratory virus, for example, might provoke a healing response that continues in an unregulated manner.

Why is IPF in women on the rise? The reasons may include that experts are getting better at detecting the disease. They are also getting a better understanding of the role genes play in IPF. Sometimes, members of the same family develop IPF, which indicates a genetic component to the disease.

There is no proven treatment for IPF other than lung transplantation. Many drugs tested have failed to demonstrate a benefit. Pirfenidone, a drug that failed to show a clear benefit in a large clinical trial, did show some promise and will be tested again. Another drug, BIBF, has shown considerable promise in a recent trial. Another trial testing BIBF will start soon. Trials of other drugs are also underway to find effective treatments for IPF.

Dr. Neil Ettinger is a pulmonologist at St. Luke's Hospital. To participate in a clinical trial for the treatment of IPF, call 314-439-LUNG (5864) or visit the CardioPulmonary Research Center page.

This article was published in the St. Louis Post-Dispatch on December 1, 2011.