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.
Shortness of breath during activity (this symptom lasts for months or years, and over time may also occur when at rest)
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.
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:
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.
Avoiding smoking may help prevent this condition, but how to prevent the cause is not known.
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).
Review Date: 4/27/2010 Reviewed 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.