Pulmonary fibrosis is the formation or development of excess fibrous connective tissue in the lungs. Pulmonary fibrosis is one of a family of related diseases called interstitial lung diseases. All of these diseases can result in lung scarring. (Ebina M 2010) "Fibrosis" is a term used to refer to scarring, so pulmonary fibrosis means scarring throughout the lungs. In some people, chronic pulmonary inflammation and fibrosis develop without an identifiable cause. Most of these people have a condition called idiopathic pulmonary fibrosis (IPF) that does not respond to medical therapy, while some of the other types of fibrosis, such as nonspecific interstitial pneumonitis (NSIP), may respond to immune suppressive therapy. (Welsh M.J. 2010) Tissue deep in the lungs and between the air sacs become thick, stiff and scarred, as the lung tissue becomes scarred; it interferes with a person's ability to breathe. This is because it is harder for oxygen to pass through the walls of the air sac into the bloodstream. In some cases, the cause of pulmonary fibrosis can be found. But most cases of pulmonary fibrosis have no known cause. Pulmonary fibrosis can develop slowly or quickly. There is no cure, once the lung tissue becomes scarred, the damage cannot be reversed. Many people with the disease live only about three to five years after diagnosis. About 140,000 Americans have been diagnosed with pulmonary fibrosis. It is most likely to affect people ages 50 to 75. Pulmonary fibrosis can lead to other medical problems, including collapsed lung, lung infections, blood clots in the lungs and lung cancer. As the disease gets worse, it can lead to respiratory failure, pulmonary hypertension and heart failure. A person with pulmonary fibrosis may need supplemental oxygen to help with breathing. The condition is believed to result from an inflammatory response to an unknown substance. Pulmonary fibrosis can be caused by many conditions including chronic inflammatory processes,...
References: Am J Respir Crit Care Med. 2000; American Thoracic Society (ATS) and European Respiratory Society (ERS). Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement. 161:646-664.
Fernandez Perez ER. Incidence, Prevalence, and Clinical Course of Idiopathic Pulmonary Fibrosis: A Population-Based Study. Chest. Jan 2010;137:129-37.
Noth I, Martinez FJ. Recent advances in idiopathic pulmonary fibrosis. Chest. 2007; 132(2).
Nukiwa T. Lymphat Res Biol. 2010 Dec;8(4):199-207.
Please join StudyMode to read the full document