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Idiopathic pulmonary fibrosis is a chronic, progressive lung condition of unknown cause that affects more than 100,000 people in the U.S. It consists of fibrosis -- a buildup of fibrous scar tissue -- that eventually robs the lungs of the ability to transport oxygen to the bloodstream. Although the disease progresses at variable rates, most patients die within five years after being diagnosed, according to the National Institutes of Health.
"At present, there is no known cure for this devastating condition," said Paul Noble, MD, professor of Medicine and chair of the Department of Medicine, director of the Women's Guild Lung Institute and the Vera and Paul Guerin Family Distinguished Chair in Pulmonary Medicine at Cedars-Sinai. "Current FDA- approved drugs only slow the fibrosis in certain individuals or treat some symptoms. This study opens a pathway for developing a treatment for idiopathic pulmonary fibrosis."
Dianhua - Jiang - MD - PhD - Professor
Noble and Dianhua Jiang, MD, PhD, professor of Medicine at Cedars-Sinai, were co-corresponding authors of the research published in the journal JCI Insight.
The study focused on cells known as fibroblasts, which produce proteins to help build the extracellular matrix -- a meshwork of macromolecules that provides structure and biochemical support for cells in the body's tissues. In idiopathic pulmonary fibrosis, defective fibroblasts invade normal lung tissue and generate excessive deposits of fibrous (scar) tissue that progressively impairs lung function.
Lung - Tissue - Samples
In examining lung tissue samples from...
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