Idiopathic Pulmonary Fibrosis (IPF): An Overview

Shaney L Barratt, Andrew Creamer, Conal Hayton, Nazia Chaudhuri

Research output: Contribution to journalReview articlepeer-review


Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterised by chronic, progressive scarring of the lungs and the pathological hallmark of usual interstitial pneumonia. Current paradigms suggest alveolar epithelial cell damage is a key initiating factor. Globally, incidence of the disease is rising, with associated high morbidity, mortality, and economic healthcare burden. Diagnosis relies on a multidisciplinary team approach with exclusion of other causes of interstitial lung disease. Over recent years, two novel antifibrotic therapies, pirfenidone and nintedanib, have been developed, providing treatment options for many patients with IPF, with several other agents in early clinical trials. Current efforts are directed at identifying key biomarkers that may direct more customized patient-centred healthcare to improve outcomes for these patients in the future.

Original languageEnglish
Article number201
Number of pages21
JournalJournal of Clinical Medicine
Issue number8
Early online date6 Aug 2018
Publication statusPublished - Aug 2018


  • idiopathic pulmonary fibrosis
  • interstitial lung disease
  • nintedanib
  • pirfenidone


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