What is the treatment of Idiopathic Pulmonary Fibrosis?

By  ,  National Institute of Health
Jan 10, 2013

Idiopathic pulmonary fibrosis (IPF) is treated with medicines, oxygen therapy, pulmonary rehabilitation (rehab), and lung transplant.

The goals of treating IPF are to prevent more lung scarring, relieve your symptoms, maintain your ability to be active and get around, keep you healthy, and improve your quality of life.
Treatment can't remove scarring that has already happened. As a result, diagnosing and treating IPF as early as possible, before a lot of scarring has taken place, is very important.
Treatment usually is based on your age, medical history, any medical problems you may have, and how much the IPF has advanced.


The main treatment for IPF is medicine to reduce inflammation. Many doctors also add a medicine to suppress your body's immune system. These treatments can prevent further scarring and increase survival time in some people, but they don't work for everyone.


The anti-inflammatory medicine that most doctors prescribe is high-dose prednisone, a corticosteroid. You usually take prednisone 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 only 3 to 6 months at first. Then, if it works for you, your doctor may reduce the dose over time and keep you on it for a longer time.

Most people who take high-dose prednisone for a long time can have side effects, including insomnia, weight gain, acne, and irritability. Using prednisone for a long time also can lead to other conditions, including:

•    High blood pressure

•    Hyperglycemia (high blood sugar)

•    Cataracts (a cloudy area on an eye's lens)

•    Glaucoma (a serious eye condition that can lead to blindness)

•    Anxiety or depression

•    Osteoporosis (thinning of the skin and bones)

•    Adrenal gland insufficiency (a condition in which the adrenal glands don't produce enough of certain hormones). This condition should be treated by an endocrinologist. This is a doctor who specializes in diagnosing and treating a number of endocrine problems, including adrenal gland problems.


Prednisone also can cause conditions such as diabetes and glaucoma to get worse.

Other Medicines

Many doctors prescribe a second medicine with prednisone.

Azathioprine. This medicine affects your immune system. Most patients 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.

Then, if you don't have serious side effects and the drug combination seems to help you, your doctor may keep you on it long term. The most common side effects of azathioprine include:

•    Nausea (feeling sick to your stomach), vomiting, diarrhea, and fever and chills

•    Anemia (a low red blood cell count) and low platelet and white blood cell counts

•    Liver problems

•    Pancreatitis or lym...

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