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Drug for Deadly Lung Disease Shows Promise
Date:5/23/2008

Idiopathic pulmonary fibrosis kills 40,000 Americans each year

FRIDAY, May 23 (HealthDay News) -- Patients with a progressive fibrosis of the lungs that's fatal within a few years of diagnosis may finally have some reason for hope.

Japanese researchers say daily use of the drug pirfenidone improved the lung function and lengthened the survival of patients with the illness, called idiopathic pulmonary fibrosis (IPF).

"Patients look to any research in IPF with a sense of hope, because right now, there's very little that can be done for them," said Mark Shreve, founder and chief operating officer of the Coalition for Pulmonary Fibrosis, based in San Jose, Calif.

"To say that there's a desperate need -- even that would be an incredible understatement, because you are talking about a devastating, relentless disease that has a survival rate of less than three years, and no proven cause and no treatment," he said.

But the results of the new phase III clinical trial, involving 275 Japanese patients with mild-to-moderate IPF, may change all that. The findings were presented this week at the annual meeting of the American Thoracic Society, in Toronto.

According to Shreve, 128,000 Americans are battling IPF at any given time. Each year, 48,000 new cases are diagnosed, and 40,000 people die from the illness -- equal to the annual death toll from breast cancer.

IPF's origins remain largely unknown. It typically arises in late middle-age or the senior years and involves a progressive fibrosis: a process in which healthy lung tissue turns into useless scar tissue. This hardening of the lungs gradually and relentlessly robs patients of their ability to breathe.

"There's no drug, period, that's ever been approved for IPF," Shreve said. "Right now, the only treatment option that's been shown to extend the lives of patients is a lung transplant. But, other than that, the disease itself is an
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