Thumbnail image of: Respiratory System: Illustration

Pulmonary Fibrosis

What is pulmonary fibrosis?

Pulmonary fibrosis is a lung disease that causes scar tissue in your lungs.

Inside the lungs are tiny air sacs, called alveoli. These sacs help the oxygen you breathe in to get into your bloodstream. The air sacs can get swollen and irritated. Over time, the sacs are replaced by scar tissue. As the disease worsens, the scar tissue gets thicker and makes it harder to breathe. There is less oxygen in your blood, so your body does not get enough oxygen to work properly.

Pulmonary fibrosis is most common in adults between the ages of 40 and 70.

What is the cause?

The exact cause of fibrosis is not always known. Problems that increase your risk for pulmonary fibrosis include:

  • Long-term or repeated lung infections
  • Radiation therapy to the chest
  • Some medical problems, such as rheumatoid arthritis
  • Long-term use of medicines that are used to treat cancer, some heart problems, certain infections, rheumatoid arthritis, lupus, or sarcoidosis
  • Long-term exposure to cigarette smoke, chemicals, or dust in jobs such as farming, mining, or construction

Pulmonary fibrosis may be inherited, which means that it is passed from parents to children through their genes. Genes are inside each cell of your body. They contain the information that tells your body how to develop and work. You may inherit the disease, or you may be born with a greater risk for getting symptoms from things such as asbestos.

What are the symptoms?

Symptoms may include:

  • A dry cough
  • Shortness of breath, at first only when you are active, but over time happens even at rest
  • Feeling tired all the time
  • Weight loss
  • Widening and rounding of the tips of the fingers or toes (clubbing)
  • Getting infections more easily, especially lung infections

How is it diagnosed?

Your healthcare provider will ask about your symptoms and medical history and examine you. Tests may include:

  • Blood tests
  • A breathing test called spirometry
  • Chest X-ray
  • CT scan, which uses X-rays and a computer to show detailed pictures of your chest
  • Bronchoscopy, which uses a flexible, lighted tube passed through your mouth and down into your lungs to see abnormal areas. Along with the bronchoscopy you may have a:
    • Bronchoalveolar lavage, which gets samples of cells from the lungs with a rinse of the airways and air sacs with a tiny amount of fluid. The cells are tested to find the cause of the fibrosis.
    • Lung biopsy, which is the removal of a small sample of lung tissue for testing

How is it treated?

There is no cure for pulmonary fibrosis. Once scar tissue has formed, the tissue cannot go back to normal. Treatments can:

  • Relieve symptoms so you can breathe and feel better.
  • Help you be more active.
  • Help prevent more scarring.

Your healthcare provider may prescribe medicines to:

  • Keep down the swelling inside your airways. You may need steroids if your symptoms are severe or if you start having symptoms more often. Steroids are usually inhaled, but they may also be taken as a pill. Using a steroid for a long time can have serious side effects.
  • Keep your immune system from being overactive. The immune system is your body’s defense against infection. These medicines can also cause serious side effects.

Take these medicines exactly as your healthcare provider prescribes. Don’t take more or less than prescribed by your provider. Don’t stop taking these medicines without your provider’s approval. You may have to lower your dosage slowly before stopping.

Your healthcare provider may also recommend:

  • Quitting smoking and making changes in your environment so you are not around things that irritate your lungs
  • Respiratory therapy to help you learn how to exercise and how to manage your disease
  • Oxygen therapy to help you feel less short of breath and more energetic

Your provider may suggest a lung transplant if you have no other medical problems, and are not being helped by other treatments. Talk with your healthcare provider about this.

How can I take care of myself?

Follow the full course of treatment prescribed by your healthcare provider. In addition:

  • If you smoke, try to quit. Talk to your healthcare provider about ways to quit smoking.
  • Avoid breathing dust and chemical fumes.
  • Try some moderate exercise, such as walking or riding a stationary bicycle. This helps you keep your strength up. It also helps keep your lungs working. Talk to your healthcare provider before you start a new exercise program.
  • Practice breathing exercises as recommended by your provider or your respiratory therapist.
  • Get a flu shot as soon as it is available in the Fall to protect against flu.
  • Ask your healthcare provider if you should get the pneumococcal shot. This shot helps prevent serious complications of pneumonia, such as an infection of the blood or brain.
  • Join a support group in your area.
  • Take care of your health. Try to get at least 7 to 9 hours of sleep each night. Eat a healthy diet and try to keep a healthy weight. If you want to drink alcohol, ask your healthcare provider how much is safe for you to drink. Learn ways to manage stress.
  • Contact your healthcare provider if you have new or worsening symptoms.

Ask your provider:

  • How and when you will hear your test results
  • How long it will take to recover
  • If there are activities you should avoid and when you can return to your normal activities
  • How to take care of yourself at home
  • What symptoms or problems you should watch for and what to do if you have them
  • Make sure you know when you should come back for a checkup.

How can I help prevent pulmonary fibrosis?

If you have a family history of pulmonary fibrosis, you may be able to decrease your risk if you

  • Do not smoke.
  • Stay away from coal dust, asbestos, and other chemicals used in farming, mining, or construction.
Developed by RelayHealth.
Adult Advisor 2015.1 published by RelayHealth.
Last modified: 2014-07-31
Last reviewed: 2014-07-31
This content is reviewed periodically and is subject to change as new health information becomes available. The information is intended to inform and educate and is not a replacement for medical evaluation, advice, diagnosis or treatment by a healthcare professional.
Copyright ©1986-2015 McKesson Corporation and/or one of its subsidiaries. All rights reserved.

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