Doctors do not know what causes idiopathic pulmonary fibrosis (IPF) or why some people get it. Idiopathic means the cause is not known. The condition may be due to the lungs responding to an unknown substance or injury. Genes may play a role in developing IPF. The disease occurs most often in people between 50 and 70 years old.
When you have IPF, your lungs become scarred and stiffened. This makes it hard for you to breathe. In some people, IPF gets worse quickly over months or a few years. In others, IPF worsens over a much longer time.
Shortness of breath during activity (this symptom lasts for months or years, and over time may also occur when at rest)
Exams and tests
The health care provider will perform a physical exam and ask about your medical history. You will be asked whether you have been exposed to asbestos and if you have been a smoker.
The physical exam may find that you have:
Abnormal breath sounds called cackles
Blue-colored skin (cyanosis) around the mouth or fingernails due to low oxygen (with advanced disease)
Enlargement of the fingernail bases, called clubbing (with advanced disease)
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:
Call your health care provider promptly if you have any of the following::
Your breathing is getting harder, faster, or shallow (are unable to take a deep breath)
Need to lean forward when sitting to breathe comfortably.
Feel sleepy or confused
Coughing up dark mucus
Fingertips or the skin around your fingernails is blue
American Thoracic Society (ATS), European Respiratory Society (ERS), Japanese Respiratory Society (JRS), and Latin American Thoracic Association (ALAT). Idiopathic pulmonary fibrosis: evidence based guideline for diagnosis and management. Am J Respir Crit Care Med. 2011; 183:788-824.
Selman M, Morrison LD, Noble PW, King TE Jr. Idiopathic interstitial pneumonias. In: Mason RJ, Broaddus VC, Martin TR, et al., eds. Murray and Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa.: Elsevier Saunders; 2010:chap 57.
Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.