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Pitt team first to profile genes in acutely ill idiopathic pulmonary fibrosis patients
Date:7/6/2009

thor Kazuhisa Konishi, M.D., a visiting postdoctoral fellow in Dr. Kaminski's lab who performed the gene profiling. "But nearly 600 genes were differentially expressed between IPF patients who had accelerated disease and those who were stable."

There was no evidence that infection or inflammation was the cause of disease acceleration, he noted. Instead, there were indications that the cells of the alveolar epithelium, which is the tissue that covers the surface of the air sacs, were rapidly dying.

"That could mean that drugs that are used to protect the epithelium in other illnesses, such as cancer, might help IPF patients survive an exacerbation," said study co-author Kevin Gibson, M.D., associate professor in the Division of Pulmonary, Allergy and Critical Care Medicine at Pitt School of Medicine and medical director of the Simmons Center. "If we can keep them alive, there's a chance they could get a life-saving lung transplant."

To test whether the changes in the lungs could be revealed in the blood, the Pitt investigators contacted Dr. Kaminski's longtime collaborator, Dong Soon Kim, M.D., a renowned IPF researcher at Asan Medical Center and the University of Ulsan in Seoul, South Korea, who has been at the forefront of studying the acceleration syndrome. With her help, they found that levels of a protein called alpha-defensin were particularly high in the blood of patients experiencing an exacerbation. If the findings are verified with more research, which is underway, the proteins could be the first biomarker blood tests that doctors could track to identify patients at risk for sudden deterioration of lung function.

"This work opens an important window into the mystery of why patients with lung fibrosis suddenly decompensate and how to identify these patients for more aggressive therapies," said Mark T. Gladwin, M.D., chief of Pitt's Division of Pulmonary, Allergy and Critical Care Medicine. "Our current research efforts i
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Contact: Anita Srikameswaran
SrikamAV@upmc.edu
412-647-3555
University of Pittsburgh Schools of the Health Sciences
Source:Eurekalert

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