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Idiopathic Pulmonary Fibrosis
i-de-o-PA-thic PUL-mo-nar-e fi-BRO-sis
by Laurie B. Rosenblum, MPH
Idiopathic pulmonary fibrosis (IPF) is a chronic disease. It causes inflammation and fibrosis (scarring) of tissue in the lungs.
Idiopathic means the cause is not known.
Researchers think that IPF is an exaggerated and uncontrolled inflammatory response. This produces the scar tissue. What starts the cycle is not known. Over time, scarring surrounds the thin walled air sacs in the lungs. This makes the tissue thicker and stiffer. As a result, breathing becomes difficult. The lungs gradually lose their ability to pass oxygen to the rest of the body.
Risk Factors TOP
IPF occurs most often in males and people aged 50 years and older. Other factors that may increase your chance of IPF include:
Over time, the symptoms get worse. This makes daily activities difficult. People with IPF gradually start to have some or all of these symptoms:
You will be asked about your symptoms and medical history. A physical exam will be done.
Your bodily fluids and tissues may be tested. This can be done with:
Images may be taken of your lungs. This can be done with:
Your lung function may be tested. This can be done with:
There is no known cure. The goal of treatment is to improve symptoms and slow the disease process. This is done by reducing inflammation and scarring. The tissue that is already scarred cannot be healed.
Medication is the main form of treatment. It does not work for everyone. Medications are used to:
Gastroesophageal Reflux Disease
If present, GERD will need to be treated. This most often involves the use of medication and lifestyle changes.
Supportive Care TOP
There is no proven way to prevent IPF. However, avoiding smoking and wearing masks for work in some occupations may help.
American Lung Association
Coalition for Pulmonary Fibrosis
Explore idiopathic pulmonary fibrosis. National Heart, Lung, and Blood Institute website. Available at:
...(Click grey area to select URL)
Updated September 20, 2011. Accessed May 9, 2016.
Idiopathic pulmonary fibrosis. American Thoracic Society. Available at: https://www.thoracic.org/patients/patient-resources/resources/idiopathic-pulmonary-fibrosis.pdf. Updated March 2015. Accessed May 9, 2016.
Idiopathic pulmonary fibrosis. EBSCO DynaMed Plus website. Available at: http://www.dynamed.... Updated September 2, 2016. Accessed September 26, 2016.
Pulmonary fibrosis. American Lung Association website. Available at:
...(Click grey area to select URL)
Accessed May 9, 2016.
Last reviewed June 2016 by Michael Woods, MD
Last Updated: 6/20/2013
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