Home / Conditions / Pulmonary Fibrosis

Pulmonary Fibrosis

What is pulmonary fibrosis?

Pulmonary fibrosis is an illness in which the air sacs of the lungs (also called the alveoli) become inflamed and are gradually replaced by scar tissue. As the disease worsens, the scar tissue makes it harder to breathe. It becomes harder for the lungs to get oxygen into the bloodstream.

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

Back to the top

How does it occur?

There are over 100 causes of pulmonary fibrosis. It may be caused by chronic infection, cancer, or long-term use of drugs. Examples of drugs that might cause it are anti-cancer drugs, hydralazine, nitrofurantoin, and drugs for rheumatoid arthritis. Other causes include long-term exposure to asbestos, beryllium, silica, and coal dust and sensitivity to proteins made by animals such as birds and rats.

Pulmonary fibrosis may be hereditary. There are two ways in which this may occur. One is that the condition is inherited and symptoms show up later in life, usually in the forties or fifties. Or children may inherit a tendency to be extra sensitive to the known causes of pulmonary fibrosis, such as asbestos. Therefore they may be more likely to develop the condition when they are exposed to these causes.

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

Back to the top

What are the symptoms?

Symptoms include:

Back to the top

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:

Back to the top

How is it treated?

The aim of treatment is to reduce the inflammation of the alveoli and stop further scarring. Once scar tissue has formed in the lungs, it cannot return to normal.

Your healthcare provider will prescribe drugs, such as prednisone, cyclophosphamide, azathioprine and colchicine. These drugs help to fight the inflammation that can lead to scarring. After taking the drugs for at least three to six months, your healthcare provider can determine if they are helping. If they are, you may continue to take them, possibly for the rest of your life. Your treatment may be stopped or changed if the treatment is not helping you or is not keeping the disease from getting worse.

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

Lung transplants 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.

Back to the top

How long do the effects last?

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

Back to the top

How can I take care of myself?

Back to the top