The lung scarring that occurs in pulmonary fibrosis can't be reversed, and no current treatment has proved effective in stopping progression of the disease. Some treatments, though, may improve symptoms temporarily or slow the disease's progress. Others help improve quality of life.
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. Adding acetylcysteine, a derivative of a natural amino acid, to prednisone may slow the disease in some people. None of these combinations has proved very effective over the long run.
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. Some people carry a canister of oxygen, making them more mobile.
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
- Nutritional counseling
- Counseling and support
Lung transplantation may be an option of last resort for younger people with severe pulmonary fibrosis who no longer benefit from other treatment options.
March 18, 2014
- Idiopathic pulmonary fibrosis. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/ipf/ipf_all.html. Accessed Oct. 24, 2013.
- Rakel RE. Textbook of Family Medicine. 8th ed. Philadelphia, Pa.: Saunders Elsevier; 2011. http://www.clinicalkey.com. Accessed Oct. 24, 2013. http://www.mdconsult.com/das/book/body/191205553-4/0/1481/0.html#. Accessed Oct. 24, 2013.
- King TE. Approach to the adult with interstitial lung disease: Clinical evaluation. http://www.uptodate.com/home. Accessed Oct. 24, 2013.
- Ferri FF. Ferri's Clinical Advisor 2014. Philadelphia, Pa.: Mosby Elsevier; 2014. https://www.clinicalkey.com. Accessed Oct. 24, 2013.
- King TE. Approach to the adult with interstitial lung disease: Diagnostic testing. http://www.uptodate.com/home. Accessed Oct. 24, 2013.
- Rosenow EC (expert opinion). Mayo Clinic, Rochester, Minn. Oct. 24, 2013.
- King TE. Treatment of idiopathic pulmonary fibrosis. http://www.uptodate.com/home. Accessed Oct. 24, 2013.
- Pulmonary rehabilitation. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/pulreh/pulreh_all.html. Accessed Oct. 24, 2013.
- Wells AU. The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF): Practical implications. Respiratory Research. 2013;14(Suppl 1):S2.
- Kenn K, et al. Pulmonary rehabilitation in patients with idiopathic pulmonary fibrosis: A review. Respiration. 2013;86:89.