IIPs are often accompanied by scarring and inflammation of the lung known as pulmonary fibrosis. Pulmonary fibrosis makes the delivery of oxygen to the body's tissues difficult and is often fatal. About one-half of patients die within the first five years of being diagnosed with idiopathic pulmonary fibrosis. The study appearing in the November 1 issue of the American Journal of Respiratory and Critical Care provides new insight into what might cause IIP and new directions for preventing these diseases.
"This study illustrates the important role that a specific environmental exposure, in this case cigarette smoking, can play in the development of this type of lung disease among people who have a specific gene," said David A. Schwartz, M.D., NIEHS Director and a lead researcher on the study. "It once again underscores why people should not smoke."
"Pulmonary fibrosis currently affects approximately 100,000 people in the United States, with an estimated 30,000 people being diagnosed each year," added Elizabeth G. Nabel, MD, NHLBI Director. "This study enhances our understanding of one form of pulmonary fibrosis, which could help lead us to strategies for genetic testing, prevention, and treatment of this devastating and complex disease."
Researchers from three sites enrolled and evaluated 111 families with a diagnosis of IIP in at least tw
Contact: NHLBI Communications Office
NIH/National Heart, Lung, and Blood Institute