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What Is Idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis (IPF) is a disease of the lungs that causes scarring (fibrosis). Fibrosis can build up so that lung function is impaired and not enough oxygen can get to body tissues. Men and women are affected equally. Most people are diagnosed at 50 to 60 years old. IPF is not contagious.

What Causes IPF?

The cause is unknown. Cigarette smoking and environmental exposure have been associated with IPF. In rare cases, heredity appears to play a part.

What Are the Symptoms of IPF?

Early symptoms include a dry cough and shortness of breath (dyspnea). Later, dyspnea becomes the major problem. Activities, such as climbing stairs, walking short distances, and dressing, become hard and sometimes almost impossible. Enlargement of the fingertips (clubbing) may develop. The body has difficulty fighting infections. In advanced stages, people may need oxygen constantly. Most people live an average of 4 to 6 years after diagnosis.

How Is IPF Diagnosed?

The health care provider may suspect the diagnosis from your symptoms and the physical exam and medical history.

The health care provider will order additional tests: chest x-ray, computed tomography (CT) of the chest, blood tests, and pulmonary (lung) function tests. Bronchoscopy and lung biopsy may also be done. In bronchoscopy, the doctor puts a long, narrow, flexible, lighted tube (bronchoscope) into the lungs.

In a biopsy, small pieces of lung are studied to see how much inflammation and fibrosis the lungs have.

How Is IPF Treated?

Most people need lifelong treatment, and a lung specialist is usually involved in care.

The goals of treatment are to reduce lung inflammation and stop fibrosis. Once scar tissue forms, the lung cannot return to normal. Medications such as prednisone are often used but have limited effect. Oxygen improves breathing. Lung transplantation is an option in people who do not respond well to other treatments and is the only therapy shown to prolong survival.

DOs and DON’Ts in Managing IPF:

  • DO try to continue your normal activities, including work and exercise but avoid overexertion.
  • DO get emotional and psychological support if you get depressed.
  • DO call your health care provider if you get short of breath or have symptoms of an infection (e.g., fever, chills, sweats, cough producing phlegm).
  • DO call your health care provider if you have side effects from medicines.
  • DO ask your health care provider if you are a candidate for lung transplantation.
  • DON’T miss health care provider appointments.
  • DON’T be afraid to ask for second opinions from lung doctors specializing in IPF.

Contact the following sources:

  • National Heart, Lung, and Blood Institute
    Tel: (800) 575-9355
  • American Lung Association
    Tel: (212) 315-8700, (800) 586-4872

Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.

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