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HEALING PROCESS FOUND TO BACKFIRE IN LUNG PATIENTS>> A mechanism in the body which typically helps a person heal from an > injury, may actually be causing patients with idiopathic pulmonary > fibrosis (IPF) to get worse, researchers at the National Institute of > Environmental Health Sciences (NIEHS), a part of the National Institutes > of Health (NIH), and their collaborators have found.>> "We identified a new mechanism that explains why some patients with IPF > get more short of breath than others, in spite of similar levels of lung > scarring," said Stavros Garantziotis, M.D., an NIEHS staff clinician and > lead author on the new paper highlighted on the cover of the Nov. 1 issue > of the "American Journal of Respiratory and Critical Care

Medicine.">> Idiopathic pulmonary fibrosis is an incurable lung disease that affects > approximately 50,000 people in the United States. In IPF, the lung tissue > becomes scarred and patients have difficulty breathing, often resulting in > death. The cause is unknown, though genes as well as environmental factors > such as smoking and exposure to metal dust particles, are thought to raise > the risk.>> In healthy individuals, the body has a way of forming new blood vessels > that can help heal an injury. For example, if you cut your finger, the > body knows to deliver nutrients and cells to the injury site to promote > wound healing. However, in patients with IPF,

although there is a healing > process that occurs, researchers say the process backfires or is disrupted > and may be doing the patients more harm than good. Garantziotis explains > that this involves a blood protein called inter-alpha-trypsin inhibitor > (IaI), which binds with a connective tissue molecule called hyaluronan to > make new blood vessels.>> In people without IPF, this produces a healing process in the lungs. But > Garantziotis says something different happens in people with IPF.>> "Instead of building healthy new tissue to heal the scarring in the lungs, > patients with higher IaI levels develop vessels that are far away from > where they should be, pushing the blood away from the lung and bypassing > the area where the body gets its oxygen, thus

causing more shortness of > breath," Garantziotis explains. Patients with IPF may suffer from low > oxygen levels and shortness of breath beyond the actual effects of lung > scarring itself.>> The researchers applied a true bench-to-bedside approach for this study. > Starting with basic research findings from in vitro cell and experimental > animal studies, they were then able to demonstrate, in patients with IPF, > that higher IaI serum levels were associated with less ability to take up > oxygen, thus worsening the patients' condition.>> The researchers say there are at least two reasons why this study is > important. First, it demonstrates for the first time the important role > that a blood circulating protein plays in lung function. Secondly, it > identifies a potential new therapeutic target for

IPF.>> In addition to the NIEHS, other collaborators on the paper include the > Angiogenesis Core Facility, National Cancer Institute, Gaithersburg, Md.; > Duke University Medical Center, Durham, N.C.; Vanderbilt University > Medical Center, Nashville, Tenn.; Institute for Molecular Science of > Medicine, Aichi Medical University, Aichi, Japan; National Jewish Medical > and Research Center, Denver; and the National Heart, Lung, and Blood > Institute, Bethesda, Md.>> The NIEHS supports research to understand the effects of the environment > on human health and is part of the NIH. For more information on > environmental health topics, please visit our website at > <http://www.niehs.nih.gov>.>> The National Institutes of Health (NIH) - The Nation's Medical Research > Agency - includes 27 Institutes and Centers and is a component of the U. > S. Department of Health and Human Services. It is the primary federal > agency for conducting and supporting basic, clinical, and translational > medical research, and it investigates the causes, treatments, and cures > for both common and rare diseases. For more information about NIH and its > programs, visit <http://www.nih.gov/>.>> The National Institutes of Health (NIH) --

The Nation's Medical Research > Agency -- includes 27 Institutes and Centers and is a component of the > U.S. Department of Health and Human Services. It is the primary federal > agency for conducting and supporting basic, clinical and translational > medical research, and it investigates the causes, treatments, and cures > for both common and rare diseases. For more information about NIH and its > programs, visit <www.nih.gov>.> ----------------------------------------> Reference: Garantziotis S, Zudaire E, Trempus CS, Hollingsworth JW, Jiang > D, Lancaster LH, E, Zhuo L, Cuttitta F, Brown KK, Noble PW, > Kimata K, Schwartz DA. Serum inter-a-trypsin inhibitor and matrix >

hyaluronan promote angiogenesis in fibrotic lung injury, "Am J Resp Crit > Care," 2008;178.>> ##>> This NIH News Release is available online at:> <http://www.nih.gov/news/health/oct2008/niehs-27.htm>.Leanne StorchExecutive DirectorPulmonary Fibrosis Foundation1332 N. Halsted, Suite 201Chicago, IL 60642-2642 P F