Breakthrough reported in fight against deadly lung disease

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Researchers are reporting a breakthrough in their efforts to defeat a little-known but deadly lung disease that affects about 100,000 Americans. Trials of two drugs slowed the disease's progression by about half during year-long trials.

Most people have never heard of  idiopathic pulmonary fibrosis, or IPF. Scientists don't know what causes the disease, which tends to affect older men, and until now there has been no treatment for it. Most die within five years of diagnosis. IPF scars the lungs, causing them to harden.

"It's a really terrible disease because you just slowly over time suffocate," said Paul Noble, chair of the Department of Medicine at Cedars Sinai Hospital.

Now Noble says the tide may be turning in the fight against IPF. He is the senior author of one study  and co-author of another that found two separate drugs slowed IPF's progression by about half during a year-long trial.

"It's really a landmark week for IPF patients," he said.

The results of Noble's trials have electrified lung doctors, patients and advocates.  The findings of the drug trial were published by the New England Journal of Medicine this week and the team presented its findings at the International Conference of the American Thoracic Society in San Diego.

The drugs used in the trials are Pirfenidone, developed by InterMune Inc., and Nintedanib, developed by Boehringer Ingelheim.

The two drugs still must be approved by the Food and Drug Administration,  but Noble is excited about this breakthrough, calling it "a major first step. It really offers hope to patients."

Advocates for people with IPF agreed.

"This is a game changer," said Teresa Barnes, vice president of patient outreach for the Coalition for Pulmonary Fibrosis.

"This is one of those moments in history when you look back and say, 'remember that first positive data,'" said Barnes, who has lost five people in her family, including her father, to IPF. "It's a big deal."

Noble and his team hope to see final FDA approval of the IPF drugs within six months. If that approval does come through, he said, there will still be more work to do.

"Ultimately, our goal is to have drugs that reverse the scarring and make people better, rather than just slow the rate of progression," he said. "But this is such a major first step. It really offers hope to patients."