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

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

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:

• Chest pain (sometimes)
• Cough (usually dry)
• Not able to be as active as before
• Shortness of breath during activity (this symptom lasts for months or years, and over time may also occur when at rest)
• Feeling faint
• Gradual weight loss

There is no known cure for IPF.

Treatment is aimed at relieving symptoms and slowing disease progression:

• Pirfenidone (Esbriet) and nintedanib (Ofev) are two medicines that treat IPF. They may help slow lung damage.
• People with low blood oxygen levels will need oxygen support at home.
• Lung rehabilitation will not cure the disease, but it can help people exercise with less difficulty breathing.

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:

• Pulmonary Fibrosis Foundation -- www.pulmonaryfibrosis.org/life-with-pf/support-groups
• American Lung Association -- www.lung.org/support-and-community/

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.

Complications of IPF may include:

• Abnormally high levels of red blood cells due to low blood oxygen levels
• Collapsed lung
• High blood pressure in the arteries of the lungs
• Respiratory failure
• Cor pulmonale (right-sided heart failure)
• Death

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

• Breathing that is harder, faster, or shallower (you are unable to take a deep breath)
• To lean forward when sitting to breathe comfortably
• Frequent headaches
• Sleepiness or confusion
• Fever
• Dark mucus when you cough
• Blue fingertips or skin around your fingernails

Published Date : January 01, 2020
Published 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.

National Heart, Lung, and Blood Institute website. Idiopathic pulmonary fibrosis. www.nhlbi.nih.gov/health-topics/idiopathic-pulmonary-fibrosis. Accessed January 13, 2020.

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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.

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