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Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature

Luba Nalysnyk, Javier Cid-Ruzafa, Philip Rotella, Dirk Esser
European Respiratory Review 2012 21: 355-361; DOI: 10.1183/09059180.00002512
Luba Nalysnyk
*United BioSource Corporation, Lexington, MA, USA, #United BioSource Corporation, London, UK, ¶Boehringer Ingelheim GmbH, Ingelheim, Germany.
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  • For correspondence: lnalysnyk@aol.com
Javier Cid-Ruzafa
*United BioSource Corporation, Lexington, MA, USA, #United BioSource Corporation, London, UK, ¶Boehringer Ingelheim GmbH, Ingelheim, Germany.
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Philip Rotella
*United BioSource Corporation, Lexington, MA, USA, #United BioSource Corporation, London, UK, ¶Boehringer Ingelheim GmbH, Ingelheim, Germany.
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Dirk Esser
*United BioSource Corporation, Lexington, MA, USA, #United BioSource Corporation, London, UK, ¶Boehringer Ingelheim GmbH, Ingelheim, Germany.
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Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial pneumonia of unknown aetiology. It is a rare disease, and its incidence and prevalence are not clear. Therefore, we sought to review the published evidence on the global epidemiology of IPF.

A comprehensive review of English language literature was performed by searching Medline and EMBASE for studies on IPF epidemiology published between January 1990 and August 2011. Studies providing quantitative data on IPF incidence and/or prevalence were identified and key data collected.

15 studies reporting on the incidence and/or prevalence of IPF were identified and summarised. IPF prevalence estimates in the USA varied between 14 and 27.9 cases per 100,000 population using narrow case definitions, and 42.7 and 63 per 100,000 population using broad case definitions. In Europe, IPF prevalence ranged from 1.25 to 23.4 cases per 100,000 population. The annual incidence of IPF in the USA was estimated at 6.8–8.8 per 100,000 population using narrow case definitions and 16.3–17.4 per 100,000 population using broad case definitions. In Europe, the annual incidence ranged between 0.22 and 7.4 per 100,000 population. IPF prevalence and incidence increase with age, are higher among males and appear to be on the increase in recent years.

IPF is an orphan disease that affects a potentially increasing number of people in Europe and the USA. The observed variability in IPF incidence and prevalence may be explained by the differences in diagnostic criteria used, case definition, study population and study design.

  • Cryptogenic fibrosing alveolitis
  • epidemiology
  • interstitial lung disease
  • usual interstitial pneumonia

Footnotes

  • Provenance

    Publication of this peer-reviewed article was supported by Boehringer Ingelheim, Germany (article sponsor, European Respiratory Review issue 126).

  • Support Statement

    This study was supported by Boehringer Ingelheim GmbH.

  • Statement of Interest

    L. Nalysnyk was an employee of United BioSource Corporation at the time of study submission; he is now an employee of Genzyme Corporation. Genzyme Corporation had no involvement in the study. J. Cid-Ruzafa and P. Rotella are employees of United BioSource Corporation. D. Esser is an employee of Boehringer Ingelheim.

  • Received March 26, 2012.
  • Accepted April 13, 2012.
  • ©ERS 2012
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Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature
Luba Nalysnyk, Javier Cid-Ruzafa, Philip Rotella, Dirk Esser
European Respiratory Review Dec 2012, 21 (126) 355-361; DOI: 10.1183/09059180.00002512

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Incidence and prevalence of idiopathic pulmonary fibrosis: review of the literature
Luba Nalysnyk, Javier Cid-Ruzafa, Philip Rotella, Dirk Esser
European Respiratory Review Dec 2012, 21 (126) 355-361; DOI: 10.1183/09059180.00002512
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