Table 2.
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