How Is Idiopathic Pulmonary Fibrosis Treated?

Doctors may prescribe medicines, oxygen therapy, pulmonary rehabilitation (PR), and lung transplant to treat idiopathic pulmonary fibrosis (IPF).

Medicines

Currently, no medicines are proven to slow the progression of IPF.

Prednisone, azathioprine (A-zah-THI-o-preen), and N-acetylcysteine (a-SEH-til-SIS-tee-in) have been used to treat IPF, either alone or in combination. However, experts have not found enough evidence to support their use.

Prednisone

Prednisone is an anti-inflammatory medicine. You usually take it by mouth every day. However, your doctor may give it to you through a needle or tube inserted into a vein in your arm for several days. After that, you usually take it by mouth.

Because prednisone can cause serious side effects, your doctor may prescribe it for 3 to 6 months or less at first. Then, if it works for you, your doctor may reduce the dose over time and keep you on it longer.

Azathioprine

Azathioprine suppresses your immune system. You usually take it by mouth every day. Because it can cause serious side effects, your doctor may prescribe it with prednisone for only 3 to 6 months.

If you don't have serious side effects and the medicines seem to help you, your doctor may keep you on them longer.

N-acetylcysteine

N-acetylcysteine is an antioxidant that may help prevent lung damage. You usually take it by mouth several times a day.

A common treatment for IPF is a combination of prednisone, azathioprine, and N-acetylcysteine. However, this treatment was recently found harmful in a study funded by the National Heart, Lung, and Blood Institute (NHLBI).

If you have IPF and take this combination of medicines, talk with your doctor. Do not stop taking the medicines on your own.

The NHLBI currently supports research to compare N-acetylcysteine treatment with placebo treatment (sugar pills) in patients who have IPF.

New Medicines Being Studied

Researchers, like those in the Idiopathic Pulmonary Fibrosis Network, are studying new treatments for IPF. With the support and guidance of the NHLBI, these researchers continue to look for new IPF treatments and therapies.

Some of these researchers are studying medicines that may reduce inflammation and prevent or reduce scarring caused by IPF.

If you're interested in joining a research study, talk with your doctor. For more information about ongoing research, go to the "Clinical Trials" section of this article.

Other Treatments

Other treatments that may help people who have IPF include the following:

• Flu and pneumonia vaccines may help prevent infections and keep you healthy.
• Cough medicines or oral codeine may relieve coughing.
• Vitamin D, calcium, and a bone-building medicine may help prevent bone loss if you're taking prednisone or another corticosteroid.
• Anti-reflux therapy may help control gastroesophageal reflux disease (GERD). Most people who have IPF also have GERD.

Oxygen Therapy

If the amount of oxygen in your blood gets low, you may need oxygen therapy. Oxygen therapy can help reduce shortness of breath and allow you to be more active.

Oxygen usually is given through nasal prongs or a mask. At first, you may need it only during exercise and sleep. As your disease worsens, you may need it all the time.

For more information, go to the Health Topics Oxygen Therapy article.

Pulmonary Rehabilitation

PR is now a standard treatment for people who have chronic (ongoing) lung disease. PR is a broad program that helps improve the well-being of people who have breathing problems.

The program usually involves treatment by a team of specialists in a special clinic. The goal is to teach you how to manage your condition and function at your best.

PR doesn't replace medical therapy. Instead, it's used with medical therapy and may include:

• Exercise training
• Nutritional counseling
• Education on your lung disease or condition and how to manage it
• Energy-conserving techniques
• Breathing strategies
• Psychological counseling and/or group support

For more information, go to the Health Topics Pulmonary Rehabilitation article.

Lung Transplant

Your doctor may recommend a lung transplant if your condition is quickly worsening or very severe. A lung transplant can improve your quality of life and help you live longer.

Some medical centers will consider patients older than 65 for lung transplants if they have no other serious medical problems.

The major complications of a lung transplant are rejection and infection. ("Rejection" refers to your body creating proteins that attack the new organ.) You will have to take medicines for the rest of your life to reduce the risk of rejection.

Because the supply of donor lungs is limited, talk with your doctor about a lung transplant as soon as possible.

For more information, go to the Health Topics Lung Transplant article.

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