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 of the lungs. It causes inflammation and scarring of lung tissue.
Idiopathic means the cause is not known.
Something starts uncontrolled inflammation. The inflammation damages tissue and leads to scarring. Over time, the scars surround the thin air sacs in the lungs. They make the sacs thicker and stiffer. Gases like oxygen will have a harder time passing through these sacs. You will have to breathe harder to get enough oxygen. The lungs will worsen until they lose their ability to pass oxygen.
IPF occurs most often in males and people aged 50 years and older. Other factors that may increase your chance of IPF include:
Symptoms get worse over time. They will make daily tasks difficult. People with IPF gradually start to have some or all of these symptoms:
You will be asked about your symptoms and past health. 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 ease symptoms. Options include:
Medicine may help to ease some symptoms. A combination of medicine may be used to:
Pirfenidone and nintedanib may help to slow fibrosis. This may slow worsening of lung function
Diet and exercise can play important roles. Education support can also help to adjust to changes. A pulmonary rehabilitation program may include:
Quit and Avoid smoking
Smoking damages lung tissue. It will make symptoms worse. Tools may help to quit smoking. Inhaling smoke from others can be harmful as well.
There is no proven way to prevent most IPF. Proper protection when working at a high risk job may help.
American Lung Association
Coalition for Pulmonary Fibrosis
Explore idiopathic pulmonary fibrosis. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health-topics/idiopathic-pulmonary-fibrosis. Accessed January 17, 2019.
Idiopathic pulmonary fibrosis. American Thoracic Society. Available at: https://www.thoracic.org/patients/patient-resources/resources/idiopathic-pulmonary-fibrosis.pdf. Accessed January 17, 2019.
Idiopathic pulmonary fibrosis. EBSCO DynaMed Plus website. Available at: http://www.dynamed... . Updated December 4, 2018. Accessed January 17, 2019.
Pulmonary fibrosis. American Lung Association website. Available at:
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Accessed January 17, 2019.
Last reviewed May 2018 by Michael Woods, MD
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