Advanced genomics and proteomics improve the diagnosis and treatment of a deadly lung disease

In an article in the Jan. 15 issue of the American Journal of Respiratory Critical Care Medicine, University of Pittsburgh researchers report that a serious, life-threatening form of pulmonary fibrosis, called idiopathic pulmonary fibrosis, lacks all the hallmarks of inflammation and is probably unnecessarily treated with anti-inflammatory drugs. Moreover, in a related study, the investigators identified a protein found in excess amounts in the lung tissue of patients with idiopathic pulmonary fibrosis, which may be a more appropriate target for therapy.

Interstitial lung disease describes a diverse set of chronic lung conditions that often have strikingly similar symptoms but different clinical courses. However, all are characterized by differing degrees of progressive scarring of lung tissue between the air sacs, or the interstitium. With repeated damage, the interstitium becomes thickened and stiff, or fibrotic, making it increasingly difficult for the individual to breathe. Some forms of interstitial lung disease, particularly idiopathic pulmonary fibrosis, which has no known cause, have a very high death rate due to respiratory failure. Effective treatment, however, is complicated by the fact that a definitive diagnosis often requires a lung biopsy.

"Unfortunately, many patients do not receive lung biopsies. As a result, about one-third of patients who come to our clinic have previously been misdiagnosed, and many have been treated with the wrong medications," explained James Dauber, M.D., medical director of the University of Pittsburgh's Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, and professor of medicine, division of pulmonary, allergy and critical care medicine.

To improve the diagnosis and treatment of interstitial lung diseases, Naftali Kaminski, M.D., director of the Simmons Center and associate professor of pathology and human genetics, Dr. Dauber, and their coworkers decided to test the effectiveness


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