Intended for healthcare professionals


Idiopathic pulmonary fibrosis

BMJ 2013; 347 doi: (Published 07 November 2013) Cite this as: BMJ 2013;347:f6579
  1. Owen J Dempsey, consultant chest physician1,
  2. David Miller, clinical lecturer2
  1. 1Grampian Interstitial Lung Disease Clinic, Aberdeen Royal Infirmary, Aberdeen AB25 2ZN, UK
  2. 2Institute of Medical Sciences, University of Aberdeen, Aberdeen, UK
  1. owen.dempsey{at}

Modest progress nearly 150 years after the condition was first described

In 1868, Flint described a respiratory condition called “chronic pneumonitis” and noted the bulbous appearance of one patient’s fingertips, later known as finger clubbing.1 This was probably the first recorded case of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis is a devastating form of interstitial lung disease of unknown cause. With a median survival of three years after diagnosis, it carries a prognosis worse than many cancers.2 At a conservative estimate, there are 5000 new cases each year in the United Kingdom, and at least 14 000 new patients diagnosed annually in the United States.3 4 5 Median age of onset is 70 years and about two thirds of patients are smokers.6 Once thought of as a chronic inflammatory condition, the disease is more likely to be a fibrotic response driven by abnormally activated alveolar epithelial cells.7

This year, the National Institute for Health and Care Excellence (NICE) published documents on the diagnosis and management of this condition and an appraisal of a new antifibrotic drug, pirfenidone, now approved for use in the UK.6 8 These documents supplement recent management guidelines.9 10 11

The NICE guidance highlights low awareness of the …

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