Figures
- Figure 1.
Age- and sex-stratified prevalence of idiopathic pulmonary fibrosis. #: narrow case definition.
- Figure 2.
Age-stratified incidence of idiopathic pulmonary fibrosis.
- Figure 3.
Age- and sex-stratified incidence of idiopathic pulmonary fibrosis. #: narrow case definition.
- 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
- Table 1. Prevalence and incidence of idiopathic pulmonary fibrosis (IPF) by geographic region
First author [ref.] Country and study period Study methods and case definition (year of estimate) Age yrs IPF prevalence per 100,000 population (95% CI) Annual IPF incidence per 100,000 population (95% CI) All Male Female All Male Female North America Coultas [9] USA, 1988–1990 Population-based ILD registry (1988–1990)
Cases were identified using medical records, pathology reports, death certificates and autopsy reports≥18 20.2 13.2 10.7 7.4 Fernández Pérez [11] USA, 1997–2005 Olmsted County historical cohort (2005)
Narrow case definition#≥50 27.9 8.8 13.38 6.08 (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, 1996–2000 Healthcare claims database (2000)
Narrow case definition+≥18 14.0 6.8 Broad case definition§ 42.7 16.3 Europe Thomeer [18] Belgium,
1992–1996Population-based ILD registry in 20 centres (1992–1996)
Biopsy proven UIP were identified as IPFAll 1.25 0.22 Kolek [14] Czech Republic, 1981–1990 Retrospective observational study in 24 centres (1990)
Diagnosis based on histology or typical clinical symptoms, radiological pattern and functional changesAll 12.1 0.94 Hodgson [12] Finland,
1997–1998Pulmonary clinic databases nationwide (29 clinics) (1997–1998)
IPF diagnosis according to ATS/ERS consensus (2000) [22]All 16–18ƒ Karakatsani [13] Greece, 2004 National survey of pulmonologists (2004)
IPF diagnosis according to ATS/ERS consensus (2000) [22]All 3.38 0.93 Von Plessen [20] Norway,
1984–1998Hospital 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)≥16 23.4##
(14.9–33.0)16.8 30.7 4.3## 4.0
(3.1–4.9)4.6
(3.7–5.6)Gribbin [7] UK, 1991–2003 THIN database (1991–2003)
IPF was diagnosed clinically or by open-lung biopsy≥40 4.6
(4.3–4.9)5.69
(5.24–6.18)3.44
(3.10–3.82)Navaratnam [8] UK, 2000–2009 THIN 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)≥40 7.44
(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.
