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Treatments and drugs
By Mayo Clinic staffThe lung scarring that occurs in pulmonary fibrosis can't be reversed, and no current treatment has proved effective in stopping the ultimate progression of the disease. Some treatments, though, may improve symptoms temporarily or slow the disease's progress. Others help improve quality of life.
Medications
Many people diagnosed with pulmonary fibrosis are initially treated with a corticosteroid (prednisone), sometimes in combination with other drugs that suppress the immune system, such as methotrexate or cyclosporine. None of these combinations has proved very effective. Adding N-acetylcysteine, a derivative of a natural amino acid, to prednisone may slow the disease in some people. And in clinical trials, the drug pirfenidone has been shown to improve lung function and prevent destruction of lung tissue.
Prednisone and other immunosuppressant medications can also cause serious side effects, including diabetes, glaucoma, reduced production of red blood cells, skin cancer and lymphoma. For that reason, treatment is usually discontinued if there's no improvement after six months. Although some people improve temporarily on immunosuppressant drugs, it's not clear why some people respond and others don't.
Lung transplantation
Lung transplantation may be an option of last resort for younger people with severe pulmonary fibrosis who haven't benefited from other treatment options. In order to be considered for a transplant, you must agree to quit smoking if you smoke, be healthy enough to undergo surgery and post-transplant treatments, be willing and able to follow the medical program outlined by the rehabilitation and transplant team, and have the patience and emotional strength and support to undergo the wait for a donor organ. The last requirement is particularly important because donor organs are in short supply.
Other treatment approaches
Other pulmonary fibrosis treatments focus on improving quality of life. They include:
- Oxygen therapy. Using oxygen can't stop lung damage, but it can make breathing and exercise easier, prevent or lessen complications from low blood-oxygen levels, and improve your sleep and sense of well-being. It can also reduce blood pressure in the right side of your heart. You're most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.
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Pulmonary rehabilitation. This is a formal program for people with chronic lung disease that includes, but goes far beyond, medical management. The aim of pulmonary rehabilitation is not only to treat a disease or even improve daily functioning, but also to help people with pulmonary fibrosis live full, satisfying lives. To that end, pulmonary rehabilitation programs focus on exercise, on teaching you how to breathe more efficiently, on education, and on emotional support and nutritional counseling.
Most often, this multifaceted approach requires a team of health care professionals that may include a doctor, nurse, rehabilitation specialist, dietitian and social worker. Programs can vary widely, however. Your doctor can usually tell you about pulmonary rehabilitation programs in your area. Or contact the American Lung Association for more information.
Treatments under investigation
A number of treatments for pulmonary fibrosis are being developed or are in clinical trials. You can find an extensive listing of clinical trials in the National Institutes of Health clinical trial database on the Web. You can also contact the National Heart, Lung, and Blood Institute for more information. If you think you might be interested in participating in a clinical trial, your doctor can help you find an appropriate program.
- Garantziotis S, et al. Serum inter-alpha-trypsin inhibitor and matrix hyaluronan promote angiogenesis in fibrotic lung injury. American Journal of Respiratory and Critical Care Medicine. 2008;178:939.
- Interstitial lung disease and pulmonary fibrosis. American Lung Association. http://www.lungusa.org/site/apps/nlnet/content3.aspx?c=dvLUK9O0E&b=4294229&ct=3052333. Accessed Jan. 13, 2008.
- King TE. Clinical advances in the diagnosis and therapy of the interstitial lung diseases. American Journal of Respiratory and Critical Care Medicine. 2005;172:26.
- What are the signs and symptoms of idiopathic pulmonary fibrosis? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_signsandsymptoms.html. Accessed Jan. 13, 2009.
- How is idiopathic pulmonary fibrosis treated? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_treatments.html, Accessed Jan, 13, 2009.
- McKay S, et al. Patient information handbook. Chicago, Ill.: American Pulmonary Fibrosis Foundation; 2004. http://www.pulmonaryfibrosis.org/patient.pdf. Accessed Jan 13, 2009.
- What causes idiopathic pulmonary fibrosis? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_causes.html. Accessed Jan. 14, 2009.
- Idiopathic pulmonary fibrosis: Diagnosis and treatment: International consensus statement. American Journal of Respiratory and Critical Care Medicine. 2000;161:646.
