The Basics
Written by the doctors and editors at UpToDate � �
What is idiopathic pulmonary fibrosis? " � Idiopathic pulmonary fibrosis (also called "IPF " �) 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. � �
People who get IPF are usually older than 40. It is a very serious illness that cannot be cured and gets worse over time. � �
Why did I get idiopathic pulmonary fibrosis? " � Doctors do not know exactly how IPF starts. The risk of IPF is greater for people who: � �
- 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? " � IPF can start very slowly, so it might not cause any symptoms at first. When it does, symptoms can include: � �
- Shortness of breath during exercise
- Dry cough
Are there tests for idiopathic pulmonary fibrosis? " � Yes. Doctors can do: � �
- 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. " � 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? " � There is no treatment to cure IPF. It usually gets worse slowly. But doctors can treat some of the symptoms. These treatments can include: � �
- Quitting smoking " � If you smoke cigarettes, the most important thing you can do is STOP SMOKING.
- Flu and pneumonia vaccines " � 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 " � As IPF gets worse, some people need to breathe oxygen from a tank they carry with them.
- Pulmonary rehab " � 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 " � Research studies of new treatments, such as medicines, that might help people with IPF
- Lung transplant " � 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.) � �
All topics are updated as new evidence becomes available and our peer review process is complete. � �
This topic retrieved from UpToDate on: Nov 04, 2014. � �
Topic 83031 Version 4.0 � �
Release: 22.8 - C22.208 � �
� � 2014 UpToDate, Inc. All rights reserved. � �
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