Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP
Idiopathic pulmonary fibrosis (IPF) is scarring or thickening of the lungs without a known cause.
Health care providers do not know what causes IPF or why some people develop 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 60 and 70 years old. IPF is more common in men than women.
When you have IPF, your lungs become scarred and stiffened. This makes it hard for you to breathe. In most people, IPF gets worse quickly over months or a few years. In others, IPF worsens over a much longer time.
Symptoms may include any of the following:
The provider will do a physical exam and ask about your medical history. You will be asked whether you have been exposed to asbestos or other toxins and if you have been a smoker.
The physical exam may find that you have:
Tests that help diagnose IPF include the following:
There is no known cure for IPF.
Treatment is aimed at relieving symptoms and slowing disease progression:
Making home and lifestyle changes can help manage breathing symptoms. If you or any family members smoke, now is the time to stop.
A lung transplant may be considered for some people with advanced IPF.
You can ease the stress of illness by joining a support group. Sharing with others who have common experiences and problems can help you not feel alone.
More information and support for people with IPF and their families can be found at:
IPF may improve or stay stable for a longtime with or without treatment. Most people get worse, even with treatment.
Complications of IPF may include:
Call your provider right away if you have any of the following:
National Heart, Lung, and Blood Institute website. Idiopathic pulmonary fibrosis. www.nhlbi.nih.gov/health-topics/idiopathic-pulmonary-fibrosis. Accessed January 13, 2020.
Raghu G, Martinez FJ. Interstitial lung disease. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 86.
Raghu G, Rochwerg B, Zhang Y, et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192(2):e3-e19. PMID: 26177183 pubmed.ncbi.nlm.nih.gov/26177183/.
Ryu JH, Selman M, Colby TV, King TE. Idiopathic interstitial pneumonias. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 63.
Silhan LL, Danoff SK. Nonpharmacologic therapy for idiopathic pulmonary fibrosis. In: Collard HR, Richeldi L, eds. Interstitial Lung Disease. Philadelphia, PA: Elsevier; 2018:chap 5.BACK TO TOP
Review Date: 1/1/2020
Reviewed By: Denis Hadjiliadis, MD, MHS, Paul F. Harron, Jr. Associate Professor of Medicine, Pulmonary, Allergy, and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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