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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 over the long run. Adding N-acetylcysteine, a derivative of a natural amino acid, to prednisone may slow the disease in some people.
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
- Reduce blood pressure in the right side of your heart
- Improve your sleep and sense of well-being
You're most likely to receive oxygen when you sleep or exercise, although some people may use it round-the-clock.
Pulmonary rehabilitation
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:
- Physical exercise, to improve your endurance
- Breathing techniques that improve lung efficiency
- Emotional support
- Nutritional counseling
Surgery
Lung transplantation may be an option of last resort for younger people with severe pulmonary fibrosis who haven't benefited from other treatment options.
- Idiopathic pulmonary fibrosis. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_all.html. Accessed Jan. 20, 2011.
- Rust G. Interstitial lung diseases. In: Rakel RE. Textbook of Family Medicine. 7th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/191205553-4/0/1481/0.html#. Accessed Jan. 20, 2011.
- King TE. Approach to the adult with interstitial lung disease: Clinical evaluation. http://www.uptodate.com/home/index.html. Accessed Jan. 20, 2011.
- Blundin M. Idiopathic pulmonary fibrosis. In: Ferri FF. Ferri's Clinical Advisor 2011: Instant Diagnosis and Treatment. Philadelphia, Pa.: Mosby Elsevier; 2011. http://www.mdconsult.com/books/about.do?eid=4-u1.0-B978-0-323-05610-6..C2009-0-38600-6--TOP&isbn=978-0-323-05610-6&about=true&uniqId=230100505-53. Accessed Jan. 20, 2011.
- King TE. Approach to the adult with interstitial lung disease: Diagnostic testing. http://www.uptodate.com/home/index.html. Accessed Jan. 20, 2011.
- Rosenow EC (expert opinion). Mayo Clinic, Rochester, Minn. Jan. 25, 2011.
- King TE. Treatment of idiopathic pulmonary fibrosis. http://www.uptodate.com/home/index.html. Accessed Jan. 20, 2011.
- Pulmonary rehabilitation. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/pulreh/pulreh_all.html. Accessed Jan. 20, 2011.
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