What are the incidence and prevalence of IPF?

IPF is a rare disease, affecting only a small proportion of the population. A rare disease can be classified as such if it affects fewer than 200 000 people in the US1 or fewer than 1 in 2 000 people in Europe.2

There are no reliable, large-scale data sets on the incidence or prevalence of IPF. However, several small-scale studies3-7 (see table below) have suggested that the:

  • Incidence (number of new cases each year) is 5 – 10 people per 100,000 population.
  • Prevalence (number of people suffering at any one time) is 14 – 43 people per 100,000 population.

However, the true number of patients suffering from this disease is thought to be higher as IPF is underdiagnosed and often misdiagnosed.8

While these incidence and prevalence estimates give a useful guide, care must be taken in interpreting the data as different populations were used in each study, and estimates vary considerably depending on the definition applied.

The incidence of IPF is increasing, with incidence in the UK more than doubling between 1990 and 20033 and incidence in the USA predicted to double between 2005 and 2030.6 These figures take into account the expected increase in population sizes as well as the gradual ageing of the population, so the reasons for this apparent rise are not yet understood.

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  1. US Food and Drug Administration. Definition of Disease Prevalence for Therapies Qualifying under the Orphan Drug Act. Available at: http://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/Drugs
    . Accessed May 4, 2015.
  2. European Medicines Agency. Committee for Orphan Medical Products. 2007. Available at: http://www.ema.europa.eu/docs/en_GB/document_library/Other/2010/01/WC500069805.pdf. Accessed May 4, 2015.
  3. Gribbin J, et al. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax 2006;61:980–985.
  4. Navaratnam V, et al. The rising incidence of idiopathic pulmonary fibrosis in the U.K. Thorax 2011;66:462–467.
  5. Raghu G, et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006;174:810–816.
  6. Fernández Pérez ER, et al. Incidence, prevalence, and clinical course of idiopathic pulmonary fibrosis: a population-based study. Chest 2010;137:129–137.
  7. Coultas DB, et al. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med 1994;150:967–972.
  8. Meltzer EB, et al. Idiopathic pulmonary fibrosis. Orphanet J Rare Dis 2008;3:8.

Related video

Ulrich Costabel discusses the prevalence and incidence of IPF

Ulrich Costabel discusses the prevalence and incidence of IPF