Overview

Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, you become progressively more short of breath.

The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can't pinpoint what's causing the problem. When a cause can't be found, the condition is termed idiopathic pulmonary fibrosis.

The lung damage caused by pulmonary fibrosis can't be repaired, but medications and therapies can sometimes help ease symptoms and improve quality of life. For some people, a lung transplant might be appropriate.

Pulmonary fibrosis care at Mayo Clinic

Sept. 23, 2016
References
  1. What is idiopathic pulmonary fibrosis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/idiopathic-pulmonary-fibrosis. Accessed June 22, 2016.
  2. Ferri FF. Idiopathic pulmonary fibrosis. In: Ferri's Clinical Advisor 2016. Philadelphia, Pa.: Mosby Elsevier; 2016. https://www.clinicalkey.com. Accessed April 13, 2016.
  3. Idiopathic pulmonary fibrosis. Merck Manual Professional Version. http://www.merckmanuals.com/professional/pulmonary-disorders/interstitial-lung-diseases/idiopathic-pulmonary-fibrosis. Accessed May 3, 2016.
  4. King TE. Approach to the adult with interstitial lung disease: Clinical evaluation. http://www.uptodate.com/home. Accessed April 13, 2016.
  5. King TE. Approach to the adult with interstitial lung disease: Diagnostic testing. http://www.uptodate.com/home. Accessed April 13, 2016.
  6. What is pulmonary hypertension? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/pah. Accessed June 9, 2016.
  7. Klings ES. Cor pulmonale. http://www.uptodate.com/home. Accessed June 9, 2016.
  8. King TE. Role of lung biopsy in the diagnosis of interstitial lung disease. http://www.uptodate.com/home. Accessed June 9, 2016.
  9. Islam S. Flexible bronchoscopy in adults: Preparation, procedural technique, and complications. http://www.uptodate.com/home. Accessed June 9, 2016.
  10. King TE. Treatment of idiopathic pulmonary fibrosis. http://www.uptodate.com/home. Accessed April 13, 2016.
  11. Raghu G, et al. An Official ATS/ERS/JRS/ALAT clinical practice guideline: Treatment of idiopathic pulmonary fibrosis. An Update of the 2011 clinical practice guideline. American Journal of Respiratory and Critical Care Medicine. 2015;192:e3.
  12. Ryu JH, et al. Idiopathic pulmonary fibrosis: Evolving concepts. Mayo Clinic Proceedings. 2014;89:1130.
  13. Barbara Woodward Lips Patient Education Center. Idiopathic pulmonary fibrosis (IPF). Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2005.
  14. Puglisi S, et al. New perspectives on management of idiopathic pulmonary fibrosis. Therapeutic Advances in Chronic Disease. 2016;7:108.
  15. Lake FR. Interstitial lung disease in rheumatoid arthritis. http://www.uptodate.com/home. Accessed June 15, 2016.
  16. Riggin EA. Allscripts EPSi. Mayo Clinic, Rochester, Minn. April 11, 2016.
  17. Meining A. Confocal laser endomicroscopy and endocytoscopy. http://www.uptodate.com/home. Accessed June 14, 2016.
  18. Wellikoff A, et al. Probe-based confocal laser endomicroscopy imaging of interstitial lung disease. American Journal of Respiratory and Critical Care Medicine. 2013;187:A5796.
  19. Yserbyt J, et al. Perspectives using probe-based confocal laser endomicroscopy of the respiratory tract. Swiss Medical Weekly. 2013;143:w13764.
  20. Scott JP (expert opinion). Mayo Clinic, Rochester, Minn. July 21, 2016.
  21. Walsh SL, et al. Interobserver agreement for the ATS/ERS/JRS/ALAT criteria for a UIP pattern on CT. Thorax. 2016;71:45.
  22. Sista RR (expert opinion). Mayo Clinic, Scottsdale, Arizona. July 22, 2016.