Alternative Names
Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
Definition of Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.
Causes, incidence, and risk factors
No one knows what causes pulmonary fibrosis or why some people get it. It causes the lungs to become scarred and stiffened. This stiffening may make 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.
Signs and tests
The health care provider will perform a physical exam and ask questions about your medical history. Your doctor will ask whether you have been exposed to asbestos and if you have been a smoker.
Treatment
No known cure exists for idiopathic pulmonary fibrosis. Unfortunately, no medication has been shown to improve the outcome of patients with this condition.
Expectations (prognosis)
Some patients may improve when they are treated with corticosteroids or cytotoxic drugs, but in most people the disease can get worse even with treatment. This worsening can happen quickly, or very slowly.
Review
Allen J. Blaivas, DO, Clinical Assistant Professor of Medicine UMDNJ-NJMS, Attending Physician in the Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Veterans Affairs, VA New Jersey Health Care System, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 4/27/2010
