Scientists at Johns Hopkins identified the genetic culprits that trigger a hereditary form of a fatal lung disease — idiopathic pulmonary fibrosis.
Their findings, published in the March 29, 2007, issue of the New England Journal of Medicine, may guide diagnosis and treatment for families that inherit genes for the disease, as well as for those that develop non-inherited forms.
The disease takes its names from fibrosis, or scarring of the lung tissue, which restricts lung function over time. Most people live three to five years after contracting the disease and there are no known approved treatments.
About 200,000 Americans have it, according to the National Institutes of Health. About 50,000 new cases are diagnosed each year, affecting mostly people 50 to 75 years of age.