Autoimmune hepatitis (AIH) occurs globally in people of all aged and ethnicities. One meta-analysis of 22 studies found a pooled worldwide incidence of 1.37 per 100,000 persons and prevalence of 17.44 per 100,000 persons, with a high level of heterogeneity between studies.[4]Lv T, Li M, Zeng N, et al. Systematic review and meta-analysis on the incidence and prevalence of autoimmune hepatitis in Asian, European, and American population. J Gastroenterol Hepatol. 2019 Oct;34(10):1676-84.
http://www.ncbi.nlm.nih.gov/pubmed/31146297?tool=bestpractice.com
Sub-group analysis suggested that incidence rates were similar worldwide, but prevalence was lower in Asian than in European and American populations; this difference may be due to genetic factors, as Europeans and North American populations are mainly white, with a high frequency of human leukocyte antigen (HLA)-DR3 and HLA-DR4 markers. An increase in incidence of type 1 AIH, which seems to represent a true increase of the disease, has been reported in Denmark, rising from 1.37 to 2.33 cases per 100,000 individuals per year between 1994 and 2014. This increase was also reflected by an increase in prevalence.[5]Grønbæk L, Vilstrup H, Jepsen P. Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study. J Hepatol. 2014 Mar;60(3):612-7.
http://www.ncbi.nlm.nih.gov/pubmed/24326217?tool=bestpractice.com
Women are more frequently affected than men.[5]Grønbæk L, Vilstrup H, Jepsen P. Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study. J Hepatol. 2014 Mar;60(3):612-7.
http://www.ncbi.nlm.nih.gov/pubmed/24326217?tool=bestpractice.com
[6]Ngu JH, Bechly K, Chapman BA, et al. Population-based epidemiology study of autoimmune hepatitis: a disease of older women? J Gastroenterol Hepatol. 2010 Oct;25(10):1681-6.
http://www.ncbi.nlm.nih.gov/pubmed/20880179?tool=bestpractice.com
[7]van Gerven NM, Verwer BJ, Witte BI, et al. Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands. Scand J Gastroenterol. 2014 Oct;49(10):1245-54.
http://www.ncbi.nlm.nih.gov/pubmed/25123213?tool=bestpractice.com
[8]Radhakrishnan KR, Alkhouri N, Worley S, et al. Autoimmune hepatitis in children--impact of cirrhosis at presentation on natural history and long-term outcome. Dig Liver Dis. 2010 Oct;42(10):724-8.
http://www.ncbi.nlm.nih.gov/pubmed/20163994?tool=bestpractice.com
[9]Deneau M, Jensen MK, Holmen J, et al. Primary sclerosing cholangitis, autoimmune hepatitis, and overlap in Utah children: epidemiology and natural history. Hepatology. 2013 Oct;58(4):1392-400.
https://aasldpubs.onlinelibrary.wiley.com/doi/10.1002/hep.26454
http://www.ncbi.nlm.nih.gov/pubmed/23686586?tool=bestpractice.com
[10]Jiménez-Rivera C, Ling SC, Ahmed N, et al. Incidence and characteristics of autoimmune hepatitis. Pediatrics. 2015 Nov;136(5):e1237-48.
http://www.ncbi.nlm.nih.gov/pubmed/26482664?tool=bestpractice.com
Incidence rates for women in Europe are 1.2 to 3.05 per 100,000 population, compared with 0.26 to 0.89 per 100,000 population for men.[11]Trivedi PJ, Hirschfield GM. Recent advances in clinical practice: epidemiology of autoimmune liver diseases. Gut. 2021 Oct;70(10):1989-2003.
http://www.ncbi.nlm.nih.gov/pubmed/34266966?tool=bestpractice.com
Type 1 AIH is the most common form; 78% of patients are women (female:male ratio is 4:1).[12]Moy L, Levine J. Autoimmune hepatitis: a classic autoimmune liver disease. Curr Probl Pediatr Adolesc Health Care. 2014 Dec;44(11):341-6.
http://www.ncbi.nlm.nih.gov/pubmed/25466500?tool=bestpractice.com
Type 1 AIH demonstrates a bimodal age distribution, most frequently affecting people between the aged of 10 and 30 years and 40 and 60 years.[13]Czaja AJ. Autoimmune hepatitis: approach to diagnosis. MedGenMed. 2006 May 23;8(2):55.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1785222
http://www.ncbi.nlm.nih.gov/pubmed/16926794?tool=bestpractice.com
Older peak aged of onset of AIH (>60 years) have been reported in Denmark and New Zealand.[5]Grønbæk L, Vilstrup H, Jepsen P. Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study. J Hepatol. 2014 Mar;60(3):612-7.
http://www.ncbi.nlm.nih.gov/pubmed/24326217?tool=bestpractice.com
[6]Ngu JH, Bechly K, Chapman BA, et al. Population-based epidemiology study of autoimmune hepatitis: a disease of older women? J Gastroenterol Hepatol. 2010 Oct;25(10):1681-6.
http://www.ncbi.nlm.nih.gov/pubmed/20880179?tool=bestpractice.com
Type 2 AIH mainly affects children; again, the majority of patients are female.[3]Krawitt EL. Autoimmune hepatitis. N Engl J Med. 2006 Jan 5;354(1):54-66.
http://www.ncbi.nlm.nih.gov/pubmed/16394302?tool=bestpractice.com
[13]Czaja AJ. Autoimmune hepatitis: approach to diagnosis. MedGenMed. 2006 May 23;8(2):55.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1785222
http://www.ncbi.nlm.nih.gov/pubmed/16926794?tool=bestpractice.com
[14]Mieli-Vergani G, Vergani D, Czaja AJ, et al. Autoimmune hepatitis. Nat Rev Dis Primers. 2018 Apr 12;4:18017.
http://www.ncbi.nlm.nih.gov/pubmed/29644994?tool=bestpractice.com
Prevalence is unknown. In one study in Canada that included 159 children and adolescents with AIH, the annual incidence of AIH was 0.23 cases per 100,000 children; type 1 AIH was diagnosed 5.5 times more frequently than type 2 AIH.[10]Jiménez-Rivera C, Ling SC, Ahmed N, et al. Incidence and characteristics of autoimmune hepatitis. Pediatrics. 2015 Nov;136(5):e1237-48.
http://www.ncbi.nlm.nih.gov/pubmed/26482664?tool=bestpractice.com