What is idiopathic pulmonary fibrosis (IPF)?

Updated: Aug 06, 2018
  • Author: Ali Nawaz Khan, MBBS, FRCS, FRCP, FRCR; Chief Editor: Eugene C Lin, MD  more...
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Answer

Answer

Idiopathic pulmonary fibrosis (IPF) is defined as a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown etiology, occurring primarily in older adults, limited to the lungs, and associated with the histopathologic and/or radiologic pattern of usual interstitial pneumonia (UIP). It is characterized by progressive worsening of dyspnea and lung function and is associated with a poor prognosis; most patients die of respiratory failure. [1]  The mean survival is approximately 4 years.


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