Treatment

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 may improve symptoms temporarily or slow the disease's progression. Others may help improve quality of life. Doctors will evaluate the severity of your condition to determine the most appropriate treatment for your condition.

Medications

Your doctor may recommend newer medications, including pirfenidone (Esbriet) and nintedanib (Ofev). These medications may help slow the progression of idiopathic pulmonary fibrosis. Both medications have been approved by the Food and Drug Administration (FDA). Additional medications and new formulations of these medications are being developed but have not yet been FDA approved.

Nintedanib can cause side effects such as diarrhea and nausea. Side effects of pirfenidone include rash, nausea and diarrhea.

Researchers continue to study medications to treat pulmonary fibrosis.

Doctors may recommend anti-acid medications to treat gastroesophageal reflux disease (GERD), a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis.

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 may receive oxygen when you sleep or exercise, although some people may use it all the time. Some people carry a canister of oxygen, making them more mobile.

Pulmonary rehabilitation

Pulmonary rehabilitation can help you manage your symptoms and improve your daily functioning. Pulmonary rehabilitation programs focus on:

  • Physical exercise to improve your endurance
  • Breathing techniques that may improve lung efficiency
  • Nutritional counseling
  • Counseling and support
  • Education about your condition

Lung transplant

Lung transplantation may be an option for people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. However, a lung transplant can involve complications such as rejection and infection. Your doctor may discuss with you if a lung transplant may be appropriate for your condition.

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.

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