i-de-o-PA-thic PUL-mo-nar-e fi-BRO-sis
Idiopathic pulmonary fibrosis (IPF) is a chronic disease. It causes inflammation and fibrosis (scarring) of tissue in the lungs. It occurs most often in people aged 50-70.
There are various types with similar symptoms, but different response to treatment and outcomes.
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.
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IPFoccurs most often in males and people aged 50 and older. Other factors below may increase the risk of getting it:
Over time, the symptoms get worse. This makes daily activities difficult. People with IPF gradually start to have some or all of these symptoms:
The doctor will ask about your symptoms and medical history. A physical exam will be done. One or more of the following tests may be performed:
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 returned to normal.
Medication is the main form of treatment. It does not work for some people. Options may include:
GERD will need to be treated. This most often involves the use of medication and lifestyle changes.
American Lung Association
Coalition for Pulmonary Fibrosis
National Heart, Lung, and Blood Institute
The Canadian Lung Association
Bowlby, Lynn. Idiopathic pulmonary fibrosis. In: Ferri’s Clinical Advisor . 9th ed. St. Louis, MO: Mosby; 2007.
Epidemiology and risk factors. Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org/ . Accessed January 26, 2004.
Facts about idiopathic pulmonary fibrosis. National Heart, Lung, and Blood Institute website. Available at: http://www.nhlbi.nih.gov/ . Accessed January 26, 2004.
Facts about pulmonary fibrosis and interstitial lung disease. American Lung Association website. Available at: http://www.lungusa.org/ . Accessed January 26, 2004.
Raghu, Ganesh. Intersitial lung disease. In: Cecil Medicine . 23rd ed. Philadelphia, PA: Saunders; 2007.
Reporters’ guide to idiopathic pulmonary fibrosis. Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org . Accessed January 27, 2004.
Symptoms and diagnosis. Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org/ . Accessed January 26, 2004.
Treatment. Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org/ . Accessed January 26, 2004.
What is IPF? Coalition for Pulmonary Fibrosis website. Available at: http://www.coalitionforpf.org/ . Accessed January 26, 2004.
Last reviewed February 2014 by Michael Woods, MD
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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