Idiopathic Pulmonary Fibrosis
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What is IPF?
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and irreversible fibrotic disease1 of the lung of unknown cause.2 IPF is the most common form of idiopathic interstitial pneumonia and has a debilitating effect on individuals and, although the clinical course can vary, carries a poor prognosis.3,4
The pathophysiology of IPF
IPF is thought to involve repeated injury to alveolar epithelial cells, followed by abnormal wound healing. Fibrogenesis (the build-up of scar tissue) around the alveoli makes it increasingly harder to get oxygen into the bloodstream.5,6
IPF is characterised by: 5
- Reduced lung volume
- Impaired gas exchange
- Reduced lung diffusing capacity
Facts about IPF
6,000
2-5 years
50%
Up to 40%
85%
>5,000
Symptoms
Patients typically present to primary care with dyspnoea and a persistent dry cough.8,12 For all patients with unexplained chronic exertional dyspnoea, IPF should be considered. It is more common in people over 45 years old.8,13 Clinical features of IPF typically include:7,13
pneumonia (UIP)
Figure 1: Clinical features of IPF
Risk factors and causes
The cause of IPF is unknown but a number of patient-related, occupational and environmental risk factors have been identified:2,14
- Increasing age
- Gender (IPF is more prevalent in men)
- Family history of IPF
- Gastroesophageal reflux disease
- Certain viral infections, e.g. Epstein-Barr virus, hepatitis C virus
- Smoking
Incidence and prevalence
In the UK, approximately 32,000 people live with IPF, with 6,000 new cases being diagnosed each year.7
References
- Costabel U. Eur Respir Rev 2015;24(135):65–68.
- Raghu G et al. Am J Respir Crit Care Med 2018;198(5):e44–e68.
- US Food and Drug Administration (FDA). The voice of the patient: a series of reports from the US Food and Drug Administration's (FDA's) patient-focused drug development initiative: idiopathic pulmonary fibrosis. Available at: https://www.fda.gov/files/about%20fda/published/The-Voice-of-the-Patient--Idiopathic-Pulmonary-Fibrosis.pdf (accessed November 2024).
- Ley B, Collard HR, King TE. Am J Respir Crit Care Med 2011;183(4):431–440.
- Plantier L et al. Eur Respir Rev 2018;27(147):170062.
- King TE et al. Lancet 2011;378:1949–1961.
- British Lung Foundation. The battle for breath: the impact of lung disease in the UK. 2016. Available at: https://www.blf.org.uk/policy/the-battle-for-breath-2016 (accessed November 2024).
- Raghu G et al. Am J Respir Crit Care Med 2011;183(6):788–824.
- Fujimoto H et al. Clin Med Insights Circ Respir Pulm Med 2016;8(9):179–185.
- Collard HR et al. Respir Med 2007;101(6):1350–1354.
- Ryerson CJ et al. Eur Respir J 2015;46(2):512–520.
- Richeldi L, Collard HR, Jones MG. Lancet 2017;389(10082):1941–1952.
- NICE clinical guideline [CG163]. Idiopathic pulmonary fibrosis in adults: diagnosis and management. Available at: https://www.nice.org.uk/guidance/cg163 (accessed November 2024).
- British Thoracic Society (BTS). BTS ILD Registry Annual Report 2020. Available at: https://www.brit-thoracic.org.uk/document-library/quality-improvement/ild-registry/bts-ild-registry-annual-report-2020/ (accessed November 2024).
PC-GB-110729 V2 | March 2025