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Idiopathic pulmonary fibrosis

Clubbing
Respiratory system

Definition

Idiopathic pulmonary fibrosis is scarring or thickening of the lungs without a known cause.

Alternative Names

Idiopathic diffuse interstitial pulmonary fibrosis; IPF; Pulmonary fibrosis; Cryptogenic fibrosing alveolitis; CFA; Fibrosing alveolitis; Usual interstitial pneumonitis; UIP

Causes

Health care providers 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.

Symptoms

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:

Exams and Tests

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:

Treatment

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.

Support Groups

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.

These organizations are good resources for information on lung disease:

Outlook (Prognosis)

IPF may improve or stay stable for a longtime with or without treatment. Most people get worse, even with treatment.

When breathing symptoms become more severe, you and your provider should discuss treatments that prolong life , such as lung transplantation. Also discuss advance care planning.

Possible Complications

Complications of IPF may include:

When to Contact a Medical Professional

Call your provider right away if you have any of the following:

References

Raghu G. Interstitial lung disease. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 92.

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 Jul 15;192(2):e3-19. PMID: 26177183 www.ncbi.nlm.nih.gov/pubmed/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.


Review Date: 1/30/2016
Reviewed By: Denis Hadjiliadis, MD, MHS, Assistant Professor of Medicine, Pulmonary, Allergy and Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial Team.