PITTSBURGH, July 7 The first findings from a one-of-a-kind, patient-driven effort to provide lung tissue for research might help doctors predict when patients with idiopathic pulmonary fibrosis (IPF) are becoming dangerously ill and also could point the way to interventions that could sustain them until life-saving transplants can be performed.
According to senior author Naftali Kaminski, M.D., associate professor of medicine, computational biology and pathology, and director of the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Diseases at the University of Pittsburgh School of Medicine and UPMC, the research published today in the American Journal of Respiratory and Critical Care Medicine addresses a dilemma in IPF care that currently is unsolved.
"Approximately 10 percent of patients develop an acute phase that in most cases is lethal," Dr. Kaminski explained. "There has been very little understanding of the molecular basis of this syndrome, but because of the dedication of our patients and their families, we are getting closer to some answers."
For most patients, the lung-scarring disease progresses gradually and lung function slowly deteriorates. But there is neither a cure nor effective treatment, so median survival is about three years. For unknown reasons, some IPF patients experience rapid declines that cause diffuse damage of the lung alveoli, the tiny sacs where the exchange of oxygen and carbon dioxide occurs.
To better understand the molecular mechanisms of disease exacerbation or, as Dr. Kaminski puts it, acceleration, the researchers compared the gene activity profile of the lungs of eight IPF patients whose disease was dramatically worsening when they died with those of 23 stable IPF patients and 15 people with healthy lungs.
In the first analysis of its kind, "gene activity patterns were found to be more similar among all IPF patients compared to healthy people," said lead au
|Contact: Anita Srikameswaran|
University of Pittsburgh Schools of the Health Sciences