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Genetic Breakthrough Spells Hope for Lung Fibrosis Patients

Might help those with advanced disease buy time until transplant, study shows

WEDNESDAY, July 8 (HealthDay News) -- Genes that can help doctors predict when patients with idiopathic pulmonary fibrosis are becoming seriously ill have been identified by U.S. researchers, who said the findings might help keep patients alive until they can get a lung transplant.

Idiopathic pulmonary fibrosis (IPF) is a lung-scarring disease that progresses slowly and causes a gradual decline in lung function. There is no cure or effective treatment for IPF, and median survival is about three years. However, some patients experience a more rapid deterioration.

"Approximately 10 percent of patients develop an acute phase that in most cases is lethal," senior study author Dr. Naftali Kaminski said in a news release. She is director of the interstitial lung diseases center at the University of Pittsburgh School of Medicine and the University of Pittsburgh Medical Center.

In a study designed to learn more about the molecular mechanisms of accelerated IPF, the researchers compared the gene activity profile of the lungs of eight IPF patients who were experiencing dramatic worsening of their disease when they died, 23 stable IPF patients and 15 people with healthy lungs.

Differences in the expression of nearly 600 genes were noted between IPF patients with accelerated disease and those with stable disease. The researchers found no evidence that infection or inflammation was the cause of accelerated IPF. They did find signs that the cells of the alveolar epithelium, the tissue that covers the surface of the lung's air sacs, were rapidly dying.

"That could mean that drugs that are used to protect the epithelium in other illnesses, such as cancer, might help IPF patients survive an exacerbation. If we can keep them alive, there's a chance they could get a lifesaving lung transplant," study co-author Dr. Kevin Gibson, an associate professor in the pulmonary, allergy and critical care medicine division at the University of Pittsburgh School of Medicine and medical director of the interstitial lung diseases center, said in the release from the school.

The study appears in the July 15 issue of the American Journal of Respiratory and Critical Care Medicine.

More information

The U.S. National Heart, Lung, and Blood Institute has more about idiopathic pulmonary fibrosis.

-- Robert Preidt

SOURCE: University of Pittsburgh Schools of Public Health, news release, July 7, 2009

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