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Idiopathic pulmonary fibrosis

Definition

Idiopathic pulmonary fibrosis is scarring or thickening of the lungs 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

No one knows what causes pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening makes it increasingly difficult to breathe. In some people the disease gets worse quickly (over months to a few years), but other people have little worsening of the disease over time.

The condition is believed to result from an inflammatory response to an unknown substance. "Idiopathic" means no cause can be found. The disease occurs most often in people between 50 - 70 years old.

References

Goldman L, Ausiello D.Goldman: Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders; 2007.

Review Date: 1/18/2008
Reviewed By: Andrew Schriber, MD, FCCP, Specialist in Pulmonary, Critical Care, and Sleep Medicine, Virtua Memorial Hospital, Mount Holly, New Jersey. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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