| First author | Country | Publication year | Case-finding methods | Diagnostic methods | Population | Prevalence | Female prevalence | Male prevalence | Incidence | Female incidence | Male incidence |
| Rautiainen et al. [11] | Finland | 2007 | 1, 2, 5, 6 | 2/3 of a, b, d | 2,972,189 | 18.0 | 29.2 | 5.5 | 1.7 | 2.7 | 0.8 | Pla et al. [12] | Spain | 2007 | 1, 2, 3, 5 | 2/3 of a, b/c, d | 389,758 | 19.5 | 37.02 | — | 1.72 | 2.84 | — | Eaton et al. [13] | Denmark | 2007 | 2 | ICD | 5,472,032 | 12.0 | — | — | — | — | — | Baldursdottir et al. [14] | Iceland | 2012 | 1, 2, 3, 5 | 2/3 of a, b, d | 317,630 | 38.3 | 64.4 | 12.5 | 2.5 | 4.1 | 1.0 | McNally et al. [15] | England | 2014 | 1, 2, 3, 4, 5 | 2/3 of a, b, d | 2,050,000 | — | — | — | 4.509 | 7.668 | 0.949 | Koulentaki et al. [16] | Greece | 2014 | 2 | 2/3 of a, b, d | 600,000 | 36.5 | — | — | 2.088 | — | — | Boonstra et al. [17] | Netherlands | 2014 | 1, 3, 5, 6, 7 | 2/3 of a, b, d | 5,855,630 | 13.2 | — | — | 1.1 | 1.9 | 0.3 | Heetun et al. [18] | Ireland | 2015 | 2 | — | 500,000 | 10.0 | — | — | 1.4 | — | — | Lleo et al. [19] | Italy | 2016 | 2, 7 | — | 9,742,676 | 29.5 | — | — | 1.67 | 2.19 | 1.07 | Lleo et al. [19] | Denmark | 2016 | 2 | — | 5,534,738 | 12.2 | 20.3 | 3.5 | 1.14 | 1.77 | 0.47 | Gatselis et al. [20] | Greece | 2017 | 8 | 2/3 of a, b/c, d | 750,000 | 58.2 | — | — | — | — | — | Pares et al. [21] | Spain | 2018 | 1 (delphi) | 2/3 of a, b, d/US | 46,400,000 | 20.2 | — | — | 2.2 | — | — | Marzioni et al. [22] | Italy | 2019 | 2 | ICD | 1,204,216 | 27.9 | — | — | 5.31 | — | — | Madir et al. [23] | Croatia | 2019 | 2 | 2/3 of a, b, d | 331,288 | 11.5 | — | — | 0.79 | — | — | Madir et al. [23] | Croatia | 2019 | 2 | 2/3 of a, b, d | 296,195 | 12.5 | — | — | 0.89 | — | — | Marschall et al. [24] | Sweden | 2019 | 2, 4, 7 | ICD | 8,065,261 | 34.6 | — | — | 2.6 | — | — | Drazilova et al. [25] | Slovakia | 2020 | 1 | 2/3 of a, b/c, d | 1,600,000 | 14.9 | 28.0 | — | 1.2 | 2.2 | — | Sebode et al. [26] | Germany | 2020 | 7 | ICD | 8,100,000 | 36.9 | 61.2 | 12.4 | — | — | — |
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The point-prevalence rate is reported as cases per 100,000 inhabitants. The annual incidence rate is reported as new cases per 100,000 inhabitants. Cases-finding methods: (1) survey of physicians, (2) hospital records, (3) laboratory data on antimitochondrial antibody positivity, (4) death notifications, (5) histology data on liver biopsies, (6) liver transplant records, (7) pharmacy or insurance databases or billing system, and (8) prospectively collected registry. Diagnostic methods: (a) cholestatic liver panel, (b) antimitochondrial antibody positivity, (c) antinuclear (anti-gp210/anti-sp100) antibody positivity, (d) compatible liver histology, ICD: International Classification of Diseases, US: abdominal ultrasound.
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