Investigators from the Simmons Center for Interstitial Lung disease and the Division of Pulmonary, Allergy and Critical Care Medicine at the University of Pittsburgh School of Medicine will focus most of their attention on designing better methods for diagnosis and treatment of IPF. Naftali Kaminski, MD, associate professor of medicine, pathology and human genetics and director of the Simmons Center; and Kevin Gibson, MD, will follow patients to identify changes in their genes that predict disease progression. These changes may help to understand what causes the disease and to design better drugs.

“The numbers tell us that only 50 percent of patients survive three years. In reality, some of them survive much longer, while others do very badly, we currently have no way to tell,” said Dr. Gibson, associate professor of medicine. “This study should allow us to predict how the patient will do and to try and guide their options.”

One of the most devastating manifestations of IPF is the acute exacerbation of disease that leads to significant morbidity and mortality. The cause and course of these exacerbations is not completely understood. The Simmons Center study, which will monitor the changes in the expression of genes in the blood of patients over a period of five years should allow a better understanding of this phenomenon,” added Dr. Kaminski.

Steven R. Duncan, MD, associate professor of medicine, and a co-investigator, has discovered that many patients with IPF have exaggerated immune response. This new observation may suggest that not all patients will respond to the same therapy and may suggest that some patients will respond to specific drugs that modify the immune system.

“It is now accepted that you can get lung fibrosis by many mechanisms, but we still try to treat all patients the same way, and often times, we fail,” said Dr. Kaminski, who is also the director of the Lung Translational Genomics Center that aims to use the Human Genome information to understand lung disease. “This project should allow us to identify what genes are active in a specific patient during disease progression and in the future to tailor a therapy.”

Prabir Ray, PhD, associate professor of medicine has shown that certain growth factors can protect the lung against the development of fibrosis in mice. He will further study the mechanisms of these protective effects and will examine whether specific therapies could be developed. Dr. Kaminski said that Dr. Ray’s experiments are critical because they may help develop approaches not only to halt fibrosis but maybe even to reverse it.

The Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease was established five years ago through a generous donation by the Simmons family. More than a 1,000 patients with Interstitial Lung Disease are being followed up in the center; around 350 are patients with Idiopathic Pulmonary Fibrosis. In addition to providing patients access to multiple research protocols, the Center provides personal care by physicians who are experts in Interstitial Lung Diseases, information and education programs and an active support group (for more information about Simmons Center please visit http://simmonscenterild.upmc.com/.)

Source: UPMC Press Release 10/2/2006: Content edited for space