Interstitial (in-tur-STISH-ul) lung disease describes a large group of disorders characterized by progressive scarring of the lung tissue between and supporting the air sacs. The scarring associated with interstitial lung disease may cause progressive lung stiffness, eventually affecting your ability to breathe and get enough oxygen into your bloodstream.
Interstitial lung disease may be broadly categorized into known and unknown causes. Common known causes include autoimmune or rheumatologic diseases, occupational and organic exposures, medications, and radiation. Interstitial lung disease of unknown cause is predominated by idiopathic pulmonary fibrosis, a specific and progressive fibrotic lung disease, followed by the idiopathic interstitial pneumonias, such as nonspecific interstitial pneumonia (NSIP), and sarcoidosis.
Once lung scarring occurs, it's generally irreversible. Medications may slow the damage of interstitial lung disease, but many people never regain full use of their lungs. Lung transplant is an option for some people who have worsening interstitial lung disease despite treatment.
June 11, 2015
- Schraufnagel DE, et al. Breathing in America: Diseases, Progress and Hope. New York, N.Y.: American Thoracic Society; 2010. http://www.thoracic.org/education/breathing-in-america.pdf. Accessed July 8, 2014.
- Mason RJ, et al. Murray and Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa.: Saunders Elsevier; 2010. http://www.clinicalkey.com. Accessed July 8, 2014.
- King TE. Approach to the adult with interstitial lung disease: Clinical evaluation. http://www.uptodate.com/home. Accessed July 8, 2014.
- Goldman L, et al. Goldman's Cecil Medicine. 24th ed. Philadelphia, Pa.: Saunders Elsevier; 2012. http://www.clinicalkey.com. Accessed July 8, 2014.
- What is idiopathic pulmonary fibrosis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/ipf/. Accessed July 8, 2014.
- Ferri FF. Ferri's Clinical Advisor 2015: 5 Books in 1. Philadelphia, Pa.: Mosby Elsevier; 2015. https://www.clinicalkey.com. Accessed July 8, 2014.
- King TE. Treatment of idiopathy pulmonary fibrosis. http://www.uptodate.com/home. Accessed July 8, 2014.
- Pulmonary rehabilitation. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/pulreh/pulreh_all.html. Accessed July 8, 2014.
- Ryu J (expert opinion). Mayo Clinic, Rochester, Minn. July 14, 2014.
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