How Is Idiopathic Pulmonary Fibrosis Treated?

The goals of treating idiopathic pulmonary fibrosis (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 is usually based on your age, medical history, any medical problems you may have, and how much the IPF has advanced.

Treatments can include:

• Medicines to reduce inflammation (swelling) in your lungs and/or prevent more scarring
• Oxygen therapy
• Pulmonary rehabilitation
• Lung transplantation

Medicines

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.

Prednisone

The anti-inflammatory medicine that most doctors prescribe is high-dose prednisone, a corticosteroid. You usually take prednisone by mouth every day. Sometimes 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 difficulty sleeping at night (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 in the lens in your eyes).
• 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—a doctor who specializes in the diagnosis and treatment of the adrenal glands.

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

Other Medicines

Many doctors prescribe a second medicine with prednisone.

• Azathioprine (Imuran^®) is a medicine that 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, vomiting, diarrhea, and fever and chills
  • Anemia and low platelet and white blood cell counts
  • Liver problems
  • Pancreatitis or lymphoma (rarely)
• Cyclophosphamide (Cytoxan^®) is another immune system suppressant that doctors use to treat IPF. They usually add it to low doses of prednisone for patients who are getting worse while taking prednisone alone. Many patients who can't take prednisone take cyclophosphamide alone.
• Most people take cyclophosphamide by mouth every day. Some IPF patients receive it for 3 to 5 days through a needle that is inserted in a vein in the arm. After that, they take it by mouth every day. You usually start on a low dose that’s increased over time. It may take 3 to 6 months before you see any benefits from cyclophosphamide.
• The most common side effect of cyclophosphamide is a decrease in the number of blood cells that you have. This increases your chances for infection. Your doctor may order blood tests before, during, and after your treatment to see how your blood cells are affected by the drug. Other side effects can include:
  • Infertility in both men and women.
  • Nausea, diarrhea, and fatigue (tiredness).
  • Hair loss.
  • Bladder irritation. Some people who have taken cyclophosphamide for more than 2 years have developed bladder cancer. If you take cyclophosphamide, you should drink at least 8 glasses of water a day. Your doctor should test your urine at least monthly.

Other medicines that may help people with IPF include the following:

• Influenza and pneumonia vaccinations may help prevent infection and keep you healthy.
• Cough medicines or oral codeine may relieve coughing.
• Vitamin D, calcium, and a bone-building drug may help prevent bone loss if you are taking prednisone or another corticosteroid.
• Anti-reflux therapy may help control gastroesophageal reflux disease.

New Medicines Being Studied

Researchers like those in the NHLBI's Idiopathic Pulmonary Fibrosis Network are studying new treatments for IPF. Some are looking at medicines that may reduce inflammation and/or prevent or reduce scarring in IPF. These include medicines that are used to treat other conditions.

N-acetylcysteine. N-acetylcysteine is an antioxidant that’s used mostly to thin mucus in patients with other lung conditions. A recent study showed that adding it to prednisone and azathioprine helped prevent further damage to the lungs of people with IPF.

Interferon gamma-1b. Interferon gamma-1b is a manmade version of a substance that your body normally produces to help fight infections. The drug is usually injected under your skin three times a week. The most common side effects include fever, headache, muscle soreness, fatigue, and chills.

Etanercept. Etanercept is a medicine that’s used to reduce signs and symptoms of active arthritis or rheumatoid arthritis, such as joint swelling, pain, tiredness, and morning stiffness. Etanercept is also used to slow the progress of arthritis. The most common side effects are chills, cough, fever, sneezing, and sore throat.

Bosentan. Bosentan is a medicine developed to reduce high blood pressure in the blood vessels of the lungs (pulmonary arterial hypertension). You usually take this drug by mouth once or twice a day. The most common side effects include headaches, flushing, leg swelling, dizziness and fatigue, and liver problems. If you take this medicine, your doctor should test your blood often to monitor how your liver is working.

Imatinib. Imatinib is a medicine developed to treat a type of leukemia. The drug is usually given by mouth once a day. The most common side effects include fluid retention (swelling), nausea, vomiting, diarrhea, muscle cramping, and liver problems. If you take this medicine, your doctor should do frequent blood tests.

Sildenafil. Sildenafil is a medicine that is used to treat pulmonary hypertension and erectile dysfunction. It usually has few side effects.

Pirfenidone. Pirfenidone is a medicine that may decrease scarring. The drug is usually taken by mouth three times a day. The most common side effects include a rash and sun sensitivity, nausea, vomiting, loss of appetite, drowsiness, and fatigue.

Colchicine. Colchicine is a medicine that has been used to prevent or treat attacks of gout. It may slow scarring in IPF. You usually take it by mouth once or twice a day. The side effects are generally less serious than those from prednisone. They may include nausea, vomiting, stomach pain, and diarrhea.

Methotrexate. Methotrexate is an immune system suppressant that’s used to treat some cancers and autoimmune diseases. Most people take it once a week by mouth or injection. People with IPF usually take it for at least 4 to 6 months to see if it works. You may have side effects, especially if you take it at high doses.

The most serious possible side effect is liver damage. Your doctor should monitor how your liver is working every month that you’re on this drug. If you take methotrexate for more than 2 years, you may want to have a liver biopsy to make sure your liver hasn’t been damaged and that you can continue to take the medicine.

Other side effects may include nausea, mouth sores, skin rash, a decrease in infection-fighting white blood cells (requiring regular blood tests to check white cell levels), and an allergic reaction in the lungs that goes away when you stop taking the drug (this is very rare).

Your doctor should also monitor your white blood cells each month while you’re on methotrexate. You can often reduce your chances of having bad side effects from methotrexate by taking folic acid.

Penicillamine. Penicillamine is used to treat rheumatoid arthritis and to prevent kidney stones. It’s available in pill or capsule form. Several small studies have shown that penicillamine can improve lung function in some IPF patients, but more research is needed.

Penicillamine can cause serious side effects. The most common include nausea, vomiting, diarrhea, dyspepsia, and anorexia.

Cyclosporine. Cyclosporine is another immune system suppressant. It’s used mostly to prevent rejection of kidney, liver, and heart transplants. Some doctors think it may help people with IPF reduce their dose of corticosteroids while waiting for a lung transplant. More research is needed.

Oxygen Therapy

When the amount of oxygen in your blood gets low, you may need oxygen therapy to help reduce your shortness of breath and let you be more active. Oxygen is usually given through nasal prongs or a mask. At first, you may need it only during exercise and sleep. As your condition gets worse, you may need it all the time.

Pulmonary Rehabilitation

Pulmonary rehabilitation is now the standard of care for people with ongoing lung disease. It usually involves treatment by a team of specialists in a specialized clinic. The goal is to teach you how to manage your condition and function at your best.

Services usually include:

• Physical conditioning training
• Breathing exercises and retraining, so that it takes less energy for you to breathe
• Anxiety, stress, and depression management
• Nutritional counseling
• Support groups

Lung Transplantation

Early referral for surgery to replace one of your lungs with a healthy lung from a human donor is usually recommended if you:

• Are younger than 65
• Have no other medical problems
• Are not being helped by medicines

Single lung transplantation can improve your quality of life and help you live longer.

Complications can include rejection by the body of the transplanted lung and infection. You may have to take medicines for life to reduce the chances that your body will reject the transplanted lung.

Because the supply of donor lungs is limited, asking for an evaluation for a transplant as soon as possible is important.