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Alternative Names Return to topIdiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
Definition Return to top
Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.
Causes Return to top
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.
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 and 70 years old.
Symptoms Return to top
Exams and Tests Return to top
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.
Patients with idiopathic pulmonary fibrosis have abnormal breath sounds called crackles. Patients with advanced disease may have blue-colored skin (cyanosis) around the mouth or in the fingernails due to low oxygen.
Examination of the fingers and toes may show abnormal enlargement of the fingernail bases (clubbing).
Tests that help diagnose idiopathic pulmonary fibrosis include the following:
Treatment Return to top
No known cure exists for idiopathic pulmonary fibrosis. Unfortunately, no medication has been shown to improve the outcome of patients with this condition.
Some patients with advanced pulmonary fibrosis may need a lung transplant.
Support Groups Return to top
You can ease the stress of illness by joining a support group where members share common experiences and problems.
See also: Lung disease - support group
Outlook (Prognosis) Return to top
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.
Possible Complications Return to top
When to Contact a Medical Professional Return to top
Call for an appointment with your health care provider if you develop a regular cough or shortness of breath.
Prevention Return to top
Avoiding smoking may help prevent this condition, but how to prevent the cause is not known.
References Return to top
American Thoracic Society (ATS) and European Respiratory Society (ERS). Idiopathic pulmonary fibrosis: diagnosis and treatment: international consensus statement. Am J Respir Crit Care Med. 2000; 161:646-664.
Noth I, Martinez FJ. Recent advances in idiopathic pulmonary fibrosis. Chest. 2007; 132(2).Update Date: 4/24/2009 Updated by: 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.