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. 2021 Jul 7;22:197. doi: 10.1186/s12931-021-01791-z

Table 2.

IPF prevalence estimates per country

Country Study Publication year Mean unadjusted prevalence (per 10,000) Adjusted prevalence (per 10,000) 95% CI Adjusted prevalence (per 10,000) Rare disease threshold (per 10,000) Rare disease threshold met?
Asia–Pacific
 Japan Kondoh et al. [42] 2016 0.59 0.89 (0.51, 1.55) < 50,000 casesa [25] NA
Natsuizaka et al. [45] 2014 1.00
Combined 0.79
 South Korea Kim et al. [41] 2017 3.52 4.51 (2.99, 6.79) < 20,000 casesa [26] NA
Lee et al. [44] 2016 3.89
Combined 3.70
 Taiwan Lai et al. [43] 2012 0.49 0.57 (0.34, 0.94) 1 [27] Yes
Europe
 Denmark Hyldgaard et al. [39] 2014 1.01 1.17 (0.56, 2.44) 1–2 [28] Yes (upper CI out of bounds)
 Finland Kaunisto et al. [40] 2015 0.86 0.65 (0.36, 1.18) 5 [29] Yes
 France Duchemann et al. [35] 2017 0.82 0.94 (0.44, 1.99) 5 [29] Yes
 Greece Karakatsani et al. [52] 2009 0.34 0.33 (0.21, 0.53) 5 [29] Yes
 Italy Agabiti et al. [33] 2014 2.56 2.37 (1.38, 4.09) 5 [29] Yes
Harari et al. [37] 2016 2.12
Combined 2.46
 Poland Szafrański [50] 2012 2.56 2.51 (1.55, 4.05) 5 [29] Yes
 United Kingdom Strongman et al. [13] 2018 1.16 0.78 (0.38, 1.63) 5 [29] Yes
North America
 Canada Hopkins et al. [38] 2016 2.00 2.98 (1.7, 5.19) 5 [30] Yes (upper CI out of bounds)
Tarride et al. [12] 2018 7.27
Combined 2.98
 United States Fernández Pérez et al. [11] 2010 2.81 2.4 (1.33, 4.34) < 200,000a [31] Yes
Raghu et al. [47] 2014 11.1
Raghu et al. [46] 2016 0.67
Combined 1.37

CI confidence interval, IPF idiopathic pulmonary fibrosis, NA not applicable

aNumber of cases provided rather than threshold