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Definition
By Mayo Clinic staff
Pulmonary fibrosis is a serious disease that causes progressive scarring of your lung tissue.
The current thinking is that pulmonary fibrosis begins with repeated injury to the tissue within and between the tiny air sacs (alveoli) in your lungs. The damage eventually leads to scarring (fibrosis), which stiffens your lungs and makes breathing difficult. The most common symptoms are shortness of breath and a dry cough.
Current treatments for pulmonary fibrosis include medications and therapy to improve lung function and quality of life. A number of new therapies for pulmonary fibrosis are in clinical trials. In the meantime, a lung transplant may be an option for some people with pulmonary fibrosis.
- 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.
