WEDNESDAY, Sept. 21 (HealthDay News) -- An experimental drug may offer a thin ray of hope to people suffering from the rapidly fatal lung disease known as idiopathic pulmonary fibrosis.
The compound, currently known only as BIBF 1120, seems to slow the disease, decrease exacerbations and improve quality of life for patients, according to a study funded by the drug's maker, Boehringer Ingelheim, that is published in the Sept. 22 issue of the New England Journal of Medicine.
"It improves the course of disease and, in my opinion, it's the first drug to significantly ameliorate the really devastating progression of the disease," said Dr. Norman Edelman, chief medical officer for the American Lung Association, who noted that current treatments for the disease "are almost desperation attempts. There's very little evidence they work."
"The authors don't claim [BIBF 1120] is going to reverse the disease. They claim it's going to slow it down, but even that is a major factor," added Dr. Hormoz Ashtyani, director of pulmonary critical care and sleep medicine at Hackensack University Medical Center in New Jersey.
Idiopathic pulmonary fibrosis (IPF) involves a relentless stiffening of the lungs due to overproduction of collagen, the "cement" that holds lung tissue together, explained Ashtyani.
Patients with IPF usually die within two to three years of diagnosis. While the disease used to be considered relatively rare, Edelman noted that doctors have been noticing an uptick in recent years, especially among older men.
One reason researchers consider BIBF 1120 so promising is because it is "biologically plausible," said Edelman, who is also a professor of medicine at Stony Brook University in New York.
The drug inhibits tyrosine kinase receptors that are known to promote fibrosis, or lung scarring, and was successful in doing just that in an earlier study inv
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