Idiopathic Pulmonary Fibrosis
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Idiopathic Pulmonary Fibrosis

i-de-o-PA-thic PUL-mo-nar-e fi-BRO-sis

En Español (Spanish Version)

Definition

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 aged 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.

Healthy Alveoli

Alveoli

© 2008 Nucleus Medical Art, Inc.

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:

  • Sex: male
  • Age: 50 and older
  • Cigarette smoking
  • Viral infection
  • Other family members with IPF

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.

  • Shortness of breath, at first only during or after physical activity, but later also when resting
  • Dry cough
  • Gradual weight loss
  • Fatigue
  • 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:

  • Chest x-ray or CT scan—to view the lungs and check for scarring
  • Pulmonary function tests—breathing tests to measure the size and effectiveness of the lungs
  • Blood test—measurement of oxygen level in the blood to tell how well the lungs are taking up oxygen
  • Exercise test on treadmill or stationary bicycle—to measure how well the lungs and heart work during physical activity
  • Bronchoalveolar lavage—to study the cells and check for signs of inflammation. Fluid is put into the airways through a tube in the nose and mouth and then removed.
  • 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 or azathioprine to reduce the body’s immune response. High dose n-acetylcysteine is also used to improve breathing.

Some people may need to receive oxygen to help them breathe. Enrolling in a pulmonary rehabilitation program may also improve lung functioning. 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

BC Health Guide
http://www.bchealthguide.org/

The Canadian Lung Association
http://www.lung.ca

References:

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 November 2007 by Rosalyn Carson-DeWitt, 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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