Idiopathic pulmonary fibrosis (IPF) is a chronic disease that causes inflammation (swelling and irritation) and fibrosis (scarring) of tissue in the lungs. It usually occurs in people between ages 50-70.
Causes
The word “idiopathic” in the name of this disease means the cause is not known. Researchers think that IPF involves an exaggerated and uncontrolled inflammatory response that produces the fibrous scar tissue. What starts the injury and abnormal healing is not known. Over time, scarring surrounds the thin-walled air sacs in the lungs (alveoli). This makes the tissue thicker and stiffer. As a result, breathing becomes difficult, and the lungs gradually lose their ability to provide oxygen to the rest of the body.
Risk Factors
A risk factor is something that increases your chance of getting a disease or condition. Although the cause of IPF is not known, the factors below may increase the risk of getting it:
Symptoms
People with IPF gradually start to have some or all of the symptoms below. Over time, the symptoms get worse, making daily activities difficult.
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Shortness of breath, at first only during or after physical activity, but later also when resting
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Dry cough
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Gradual weight loss
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Fatigue
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Clubbing (enlargement of the fingertips or sometimes the toes)
Diagnosis
The doctor will ask about your symptoms and medical history and perform a physical exam. One or more of the following tests may be performed:
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Chest X-ray or CT scan – to view the lungs and check for scarring
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Pulmonary Function Tests – breathing tests to measure the size and effectiveness of the lungs
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Blood test – measurement of oxygen level in the blood to tell how well the lungs are taking up oxygen
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Exercise Test – on treadmill or stationary bicycle to measure how well the lungs and heart work during physical activity
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Bronchoalveolar Lavage – Fluid is put into the airways through a tube in the nose and mouth and then removed to study the cells and check for signs of inflammation.
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Lung Biopsy – a surgical procedure in which a small sample of lung tissue is removed and studied. This is usually required to confirm a diagnosis of IPF.
Treatment
IPF worsens over time, and there is no known cure. The goal of treatment is to improve symptoms and slow the disease process by reducing inflammation and scarring. The tissue that is already scarred cannot be returned to normal.
Medication is the main form of treatment, although it does not work for some people. A common combination is prednisone to reduce inflammation along with cytoxan to reduce the body’s immune response. Some people may need to receive oxygen to help them breathe. Maintaining a healthy lifestyle in terms of diet, exercise, rest, and not smoking may also help slow the disease. Lung transplantation may be considered for people with advanced IPF who do not respond to other treatment.
Prevention
There is no proven way to prevent IPF. However, avoiding smoking may help.
RESOURCES:
American Lung Association
http://www.lungusa.org
Coalition for Pulmonary Fibrosis
http://www.coalitionforpf.org
National Heart, Lung, and Blood Institute
http://www.nhlbi.nih.gov
CANADIAN RESOURCES
Canada Health Portal
http://chp-pcs.gc.ca/CHP/index_e.jsp
The Canadian Lung Association
http://www.lung.ca
References:
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.
Reporters’ guide to idiopathic pulmonary fibrosis. Coalition for Pulmonary Fibrosis website. Available at: 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.
