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)
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
    Fernández Pérez [11]USA, 1997–2005Olmsted County historical cohort (2005)
Narrow case definition#
Broad case definition63.0
    Raghu [16]USA, 1996–2000Healthcare claims database (2000)
Narrow case definition+
Broad case definition§42.716.3
    Thomeer [18]Belgium,
Population-based ILD registry in 20 centres (1992–1996)
Biopsy proven UIP were identified as IPF
    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
    Hodgson [12]Finland,
Pulmonary clinic databases nationwide (29 clinics) (1997–1998)
IPF diagnosis according to ATS/ERS consensus (2000) [22]
    Karakatsani [13]Greece, 2004National survey of pulmonologists (2004)
IPF diagnosis according to ATS/ERS consensus (2000) [22]
    Von Plessen [20]Norway,
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)
    Gribbin [7]UK, 1991–2003THIN database (1991–2003)
IPF was diagnosed clinically or by open-lung biopsy
    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)
  • 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.