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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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    Figure 1.

    Age- and sex-stratified prevalence of idiopathic pulmonary fibrosis. #: narrow case definition.

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    Figure 2.

    Age-stratified incidence of idiopathic pulmonary fibrosis.

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    Figure 3.

    Age- and sex-stratified incidence of idiopathic pulmonary fibrosis. #: narrow case definition.

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    Figure 4.

    Incidence of idiopathic pulmonary fibrosis over time. The ranges of years are represented as the final year in the range, i.e. 1997–1999 is plotted under 1999. The error bars represent 95% confidence intervals. #: narrow case definition.

Tables

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  • Table 1. Prevalence and incidence of idiopathic pulmonary fibrosis (IPF) by geographic region
    First author [ref.]Country and study periodStudy methods and case definition (year of estimate)Age yrsIPF prevalence per 100,000 population (95% CI)Annual IPF incidence per 100,000 population (95% CI)
    AllMaleFemaleAllMaleFemale
    North America
        Coultas [9]USA, 1988–1990Population-based ILD registry (1988–1990)
    Cases were identified using medical records, pathology reports, death certificates and autopsy reports
    ≥1820.213.210.77.4
        Fernández Pérez [11]USA, 1997–2005Olmsted County historical cohort (2005)
    Narrow case definition#
    ≥5027.98.813.386.08
    (10.4–45.4)(5.28–12.38)(6.51–20.24)(2.08–10.08)
    Broad case definition¶63.0
    (36.4–89.6)
    17.43
    (12.42–22.44)
    24.02
    (14.84–33.20)
    13.43
    (7.50–19.37)
        Raghu [16]USA, 1996–2000Healthcare claims database (2000)
    Narrow case definition+
    ≥1814.06.8
    Broad case definition§42.716.3
    Europe
        Thomeer [18]Belgium,
    1992–1996
    Population-based ILD registry in 20 centres (1992–1996)
    Biopsy proven UIP were identified as IPF
    All1.250.22
        Kolek [14]Czech Republic, 1981–1990Retrospective observational study in 24 centres (1990)
    Diagnosis based on histology or typical clinical symptoms, radiological pattern and functional changes
    All12.10.94
        Hodgson [12]Finland,
    1997–1998
    Pulmonary clinic databases nationwide (29 clinics) (1997–1998)
    IPF diagnosis according to ATS/ERS consensus (2000) [22]
    All16–18ƒ
        Karakatsani [13]Greece, 2004National survey of pulmonologists (2004)
    IPF diagnosis according to ATS/ERS consensus (2000) [22]
    All3.380.93
        Von Plessen [20]Norway,
    1984–1998
    Hospital records (1998)
    IPF cases identified based on physician diagnoses using ICD-8 (codes 517, 517.00, 517.01, 517.09) and ICD-9 (codes 515, 516.3, 516.8, and 516.9)
    ≥1623.4##
    (14.9–33.0)
    16.830.74.3##4.0
    (3.1–4.9)
    4.6
    (3.7–5.6)
        Gribbin [7]UK, 1991–2003THIN database (1991–2003)
    IPF was diagnosed clinically or by open-lung biopsy
    ≥404.6
    (4.3–4.9)
    5.69
    (5.24–6.18)
    3.44
    (3.10–3.82)
        Navaratnam [8]UK, 2000–2009THIN database (2000–2009)
    IPF cases identified from primary care data and death records using ICD codes (ICD-8 code 517; ICD-9 codes 516.3 and 515; ICD-10 code J84.1)
    ≥407.44
    (7.12–7.77)
    9.46
    (8.96–9.98)
    5.46
    (5.07–5.86)
    • ILD: interstitial lung disease; UIP: usual interstitial pneumonia; ATS: American Thoracic Society; ERS: European Respiratory Society; ICD: International Classification of Diseases; THIN: The Health Improvement Network. #: UIP on a surgical lung biopsy specimen or a definite UIP pattern on a high-resolution computed tomography (HRCT) image. ¶: UIP on a surgical lung biopsy specimen, or a definite or possible UIP pattern on a HRCT image. +: met broad case definition (see §) plus at least one medical claim with a procedure code for surgical lung biopsy, transbronchial lung biopsy or computed tomography of the thorax; on or before date of last medical claim with a diagnosis code for IPF. §: age ≥18 yrs, at least one medical claim with a diagnosis code for IPF (ICD-9-CM 516.3), no medical claims with a diagnosis code for any other ILDs on or after date of last medical claim with a diagnosis code for IPF. ƒ: IPF patients were estimated from evaluation, sampling and extrapolation methods combined. ##: incidence and prevalence of hospitalised IPF.

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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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