Idiopathic Pulmonary Fibrosis

The Basics

Written by the doctors and editors at UpToDate пїЅ пїЅ

What is idiopathic pulmonary fibrosis? " пїЅ Idiopathic pulmonary fibrosis (also called "IPF " пїЅ) is a lung disease that makes it hard to breathe. It damages the air sacs that send oxygen to the blood. This damage causes scars in the lungs. These changes make people with IPF cough and get short of breath. пїЅ пїЅ

People who get IPF are usually older than 40. It is a very serious illness that cannot be cured and gets worse over time. пїЅ пїЅ

Why did I get idiopathic pulmonary fibrosis? " пїЅ Doctors do not know exactly how IPF starts. The risk of IPF is greater for people who: пїЅ пїЅ

Smoke or used to smoke
Have breathed in a lot of toxic chemicals or pollution
Have breathed in certain types of dust at work, over a long time
Have family members with pulmonary fibrosis

What are the symptoms of idiopathic pulmonary fibrosis? " пїЅ IPF can start very slowly, so it might not cause any symptoms at first. When it does, symptoms can include: пїЅ пїЅ

Shortness of breath during exercise
Dry cough

Are there tests for idiopathic pulmonary fibrosis? " пїЅ Yes. Doctors can do: пїЅ пїЅ

Blood tests to look for other types of lung disease. There is no blood test for IPF.
Breathing tests to see how well your lungs are working. Breathing tests can show if your shortness of breath is caused by IPF or another disease such as emphysema.
An imaging test called a "CT scan. " пїЅ This test uses a special X-ray to create pictures of the inside of the body. It can show lung damage caused by IPF.
If the doctor is not sure you have IPF after the CT scan, he or she might do a lung biopsy. In this test, a doctor does surgery to take a small sample of tissue from your lung. Another doctor looks at the sample under a microscope for signs of IPF.

How is idiopathic pulmonary fibrosis treated? " пїЅ There is no treatment to cure IPF. It usually gets worse slowly. But doctors can treat some of the symptoms. These treatments can include: пїЅ пїЅ

Quitting smoking " пїЅ If you smoke cigarettes, the most important thing you can do is STOP SMOKING.
Flu and pneumonia vaccines " пїЅ You should get the flu shot every fall and the pneumonia vaccine at least once. Infections like the flu and pneumonia can hurt your lungs. Its important to try to prevent them.
Oxygen " пїЅ As IPF gets worse, some people need to breathe oxygen from a tank they carry with them.
Pulmonary rehab " пїЅ In pulmonary rehab, people learn exercises and ways to breathe that can help with IPF symptoms.
Treatment for acid reflux - Acid reflux is when the acid that is normally in your stomach backs up into your esophagus. The esophagus is the tube that carries food from your mouth to your stomach. Acid reflux from the esophagus can add to the lung scarring from IPF. People who have acid reflux might need medicine to stop the acid reflux from making IPF worse.
Medicine - Two medicines have been shown to slow lung damage. But they do not cure IPF.
Clinical trials " пїЅ Research studies of new treatments, such as medicines, that might help people with IPF
Lung transplant " пїЅ This is surgery to replace one or both diseased lungs with healthy lungs. It is done only if a person with IPF meets certain conditions.

IPF sometimes gets worse very quickly. If this happens, doctors can try giving antibiotics and steroid medicines. (The steroid medicine is not the same as the steroids athletes take to build muscle.) пїЅ пїЅ

All topics are updated as new evidence becomes available and our peer review process is complete. пїЅ пїЅ

This topic retrieved from UpToDate on: Nov 04, 2014. пїЅ пїЅ

Topic 83031 Version 4.0 пїЅ пїЅ

Release: 22.8 - C22.208 пїЅ пїЅ

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Idiopathic Pulmonary Fibrosis

The Basics

Consumer Information Use and Disclaimer

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