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Scientists Spot New Clues to a Deadly Lung Disease

Idiopathic pulmonary fibrosis always fatal, but research points to possible cause, potential treatment

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The findings came about after the multi-center team of researchers compared tissues and blood from patients with IPF against those of healthy patients. Those biopsies showed consistently elevated rates of CHI3L1 in the IPF group, but not in others.

"This demonstrates that the CHI3L1 plays a key role in controlling lung injury in this setting," Elias said.

The findings were further corroborated in studies in mice. The rodents were first manipulated to develop an IPF-like condition. When CHI3LI protein levels were high, the mice showed evidence of accelerated scarring of lung tissue, the team said.

While not all studies conducted in the lab or in mice translate to success in humans, the new research "lays the foundation" for efforts to develop new treatments for IPF, Elias said.

"To my knowledge this is the first comprehensive paper that's been able to explain the many facets and presentations of IPF," he added. "It explains and links the injury and the repair responses that are critical in the disease. It also provides an explanation for the slowly progressing patients and the patients that experience acute exacerbations."

The study was published June 11 in the journal Science Translational Medicine.

The news comes on the heels of two papers published in May in the New England Journal of Medicine. Those studies found that two drugs, pirfenidone and nintedanib, appear to slow the advance of IPF.

"It is an optimistic time for patients with fibrosis," Dr. Gregory Cosgrove, chief medical officer for the Pulmonary Fibrosis Foundation, said at the time of the studies' release.

"It's been frustrating when we have not identified an effective therapy over the past 10 to 15 years," he said. "But that degree of frustration has prompted the IPF community to really come together to support participation in clinical trials, and those trials have provided a foundation for these new advances."

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