Idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) is a rare form of fibrotic lung disease with no known etiology that progresses over the course of several years. It is characterized by scar tissue formation within the lungs, dyspnea, and a significantly shortened lifespan after diagnosis.

Although the etiology is unknown, cigarette smoking and certain environmental exposures have been implicated in the development of IPF.

In the absence of findings that suggest an alternative disease process, IPF can be diagnosed on clinical grounds when an appropriate history of progressive symptoms (typically dyspnea and cough) is accompanied by characteristic radiographic findings and restrictive physiology on pulmonary function testing.

Initial therapy is with an antifibrotic agent. Important supportive measures include smoking cessation, pulmonary rehabilitation, and supplemental oxygen when appropriate. Some patients may be eligible for lung transplantation.

Idiopathic pulmonary fibrosis (IPF) is a rare, chronic, life-threatening disease that manifests over several years and is characterized by the formation of scar tissue within the lungs and progressive dyspnea.[1]American Thoracic Society; European Respiratory Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. Am J Respir Crit Care Med. 2000 Feb;161(2 Pt 1):646-64. https://www.atsjournals.org/doi/full/10.1164/ajrccm.161.2.ats3-00 http://www.ncbi.nlm.nih.gov/pubmed/10673212?tool=bestpractice.com [2]Raghu G, Weycker D, Edelsberg J, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006 Oct 1;174(7):810-6. https://www.atsjournals.org/doi/full/10.1164/rccm.200602-163OC#.UmETUtglgZk http://www.ncbi.nlm.nih.gov/pubmed/16809633?tool=bestpractice.com It is the most common interstitial lung disease among the idiopathic interstitial pneumonias, which share clinical features of shortness of breath, diffuse pulmonary infiltrates on imaging, and varying degrees of inflammation, fibrosis, or both on lung biopsy.[2]Raghu G, Weycker D, Edelsberg J, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006 Oct 1;174(7):810-6. https://www.atsjournals.org/doi/full/10.1164/rccm.200602-163OC#.UmETUtglgZk http://www.ncbi.nlm.nih.gov/pubmed/16809633?tool=bestpractice.com