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Idiopathic pulmonary fibrosis

Definition:
Idiopathic pulmonary fibrosis involves scarring or thickening of tissues deep in the lung without a known cause.

Alternative Names:
Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP

Causes, incidence, and risk factors:

Idiopathic pulmonary fibrosis is a disease of the lower respiratory tract that damages the air sacs (alveoli) and leads to reduced transfer of oxygen to the blood. It causes widespread scarring of the lung.

The condition is believed to result from an inflammatory response to an unknown agent -- "idiopathic" means no cause can be found. The disease occurs most often in people between 50 and 70 years old. Nearly 75% of patients with idiopathic pulmonary fibrosis have smoked cigarettes.



Review Date: 7/17/2002
Reviewed By: David A. Kaufman, M.D., Pulmonary & Critical Care Medicine, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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