Pulmonary Fibrosis

What is pulmonary fibrosis?

Pulmonary fibrosis is a lung disease. The air sacs of the lungs get inflamed and are gradually replaced by scar tissue. (The air sacs are called the alveoli.) As the disease worsens, the scar tissue gets thicker and makes it harder to breathe. It is more difficult for for the lungs to pass the oxygen you breathe into your bloodstream.

Pulmonary fibrosis is also called interstitial lung disease. It is most common in adults between the ages of 40 and 70.

What is the cause?

There are many things that can cause pulmonary fibrosis, such as:

• long-term or repeated lung infections
• radiation therapy to the chest
• some medical problems, such as rheumatoid arthritis
• long-term use of drugs, such as:
  • anticancer drugs
  • hydralazine
  • nitrofurantoin
  • amiodarone
  • drugs for rheumatoid arthritis, lupus, and sarcoidosis

Other causes include:

• long-term exposure to asbestos, beryllium, silica, and coal dust
• sensitivity to proteins made by animals such as birds and rats

Pulmonary fibrosis may be hereditary. There are 2 ways you can get the disease because of genes you inherited from your parents:

• You inherit the disease and symptoms show up later in life, usually in the forties or fifties.
• You inherit a tendency to be extra sensitive to a substance that is known to cause the disease, such as asbestos. As a result, you may be more likely to develop the condition as a child if you are exposed to this substance.

When the cause of the fibrosis is not known, it is called idiopathic pulmonary fibrosis.

What are the symptoms?

Symptoms include:

• shortness of breath
• a dry cough
• trouble doing day-to-day activities, such as climbing stairs, walking short distances, dressing, talking on the phone, and eating
• enlarged (clubbed) fingertips
• getting infections more easily, especially lung infections.

How is it diagnosed?

Your healthcare provider will ask about your symptoms and examine you. Tests that may be used to identify pulmonary fibrosis include:

• blood tests
• a breathing test called a pulmonary function test or spirometry
• chest X-ray
• CT scan of your chest
• bronchoscopy, which is a test that uses a thin, flexible, lighted tube passed through your mouth and down into your lung to examine abnormal areas
• bronchoalveolar lavage, which gets samples of cells from the lungs with a rinse of the bronchial tubes and air sacs with a tiny amount of fluid
• lung biopsy, which is the removal of a small sample of lung tissue and may be done in 1 of 4 ways:
  • during bronchoscopy
  • by putting a needle through the chest wall between the ribs to get a piece of tissue
  • with thoracoscopy (passing tiny surgical instruments and a tiny video camera between the ribs to remove a small piece of lung)
  • with general surgery to open the chest wall and remove a small piece of lung.

For bronchoscopy, lavage, and lung biopsy you will be given an anesthetic or sedative so that you will not feel any pain during the test.

Your provider can check samples of tissue and secretions for signs of inflammation, infection, or tumors to see what might be causing your symptoms.

How is it treated?

The aim of treatment is to reduce the inflammation of the alveoli and stop more scarring. Once scar tissue has formed, the tissue cannot go back to normal.

Your healthcare provider will prescribe drugs, such as prednisone (steroids), cyclophosphamide, azathioprine, and colchicine. These drugs help to fight the inflammation that can lead to scarring. After you take the drugs for at least 3 to 6 months, your provider can determine if they are helping. If they are, you may keep taking them. You may need to take them for the rest of your life. Your treatment may be stopped or changed if the treatment is not helping you, is causing unacceptable side effects, or is not keeping the disease from getting worse.

Using a steroid for a long time can have serious side effects. Take steroid medicine exactly as your healthcare provider prescribes. Don’t take more or less of it than prescribed by your provider and don’t take it longer than prescribed. Don’t stop taking a steroid without your provider's approval. You may have to lower your dosage slowly before stopping it.

Your provider may prescribe oxygen therapy. This helps give your body the oxygen it needs. Oxygen can help you feel less short of breath and more energetic.

Lung transplants, although uncommon, may offer hope for people with severe pulmonary fibrosis and other lung diseases. Talk with your provider to see if you might be eligible for a lung transplant if a donor lung became available.

How long do the effects last?

Pulmonary fibrosis usually develops slowly, sometimes over years. The best chance of slowing the disease is to get treatment as soon as possible. You will need treatment for the rest of your life, usually from a lung specialist.

How can I take care of myself?

• Take all medicines as prescribed by your healthcare provider.
• If you smoke, stop. Avoid secondhand smoke
• Avoid areas with smog and other poor air quality--for example, smoke from any source.
• Eat a healthy diet and keep a normal weight.
• Consider eating smaller, more frequent meals. You may find it’s easier to breathe when your stomach isn't completely full.
• 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 every year.
• Get the pneumococcal pneumonia shot called Pneumovax to prevent some types of pneumonia.
• Join a support group in your community.

Call your healthcare provider if you have any questions about your condition or its treatment. If you notice anything unusual about how you are feeling or how your medicines are working, call your provider right away.

How can I help prevent pulmonary fibrosis?

Sometimes pulmonary fibrosis can be prevented by avoiding the known causes--for example, by staying away from chemical and mineral dust and particles (asbestos, coal, etc.). This is especially important if you have a family history of pulmonary fibrosis.