Autoimmune hepatitis epidemiology and demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Manpreet Kaur, MD [2]

Overview

The incidence and prevalence of autoimmune hepatitis are generally higher among individuals of the whites of northern European ancestry with a high frequency of HLA-DR3 and HLA-DR4 markers.The incidence of autoimmune hepatitis approximately 1 to 2 per 100,000 individuals worldwide. Autoimmune hepatitis has bimodal distribution usually presents around puberty and between 4th and 6th decade, although a significant proportion presents in older people usually above 65 years of age. Young women are more commonly affected by autoimmune hepatitis than men.

Epidemiology and Demographics

Incidence

The incidence of autoimmune hepatitis is approximately 1 to 2 per 100,000 individuals worldwide.
The incidence of autoimmune hepatitis is estimated to be 0.9 to 2 cases per 100,000 population annually in Europe.^[1]^[2]^[3]^[4]^[5]

Prevalence

The prevalence of autoimmune hepatitis is approximately 11 to 25 per 100,000 individuals worldwide.
In Europe, the prevalence of autoimmune hepatitis ranges from 10 to 17 per 100 000.^[6]^[7]
In Japan, the prevalence of autoimmune hepatitis ranges from 0.08-0.015 cases per 100,000 persons.

Age

Patients of all age groups may develop autoimmune hepatitis.
Autoimmune hepatitis has bimodal distribution usually presents around puberty and between 4th and 6th decade, although a significant proportion older people is usually above 65 years of age.

Race

Autoimmune hepatitis usually affects individuals of the whites of northern European ancestry with a high frequency of HLA-DR3 and HLA-DR4 markers.

Gender

Young women are more commonly affected by autoimmune hepatitis than men.
The women to men ratio is approximately 3:1.^[8]^[9]

Region

Autoimmune hepatitis is more common in North American First Nations populations compared with predominantly Caucasian, Non-first Nations populations.

References

↑ Werner M, Prytz H, Ohlsson B, Almer S, Björnsson E, Bergquist A, Wallerstedt S, Sandberg-Gertzén H, Hultcrantz R, Sangfelt P, Weiland O, Danielsson A (2008). “Epidemiology and the initial presentation of autoimmune hepatitis in Sweden: a nationwide study”. Scand. J. Gastroenterol. 43 (10): 1232–40. doi:10.1080/00365520802130183. PMID 18609163.
↑ Boberg KM, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H (1998). “Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population”. Scand. J. Gastroenterol. 33 (1): 99–103. PMID 9489916.
↑ Ngu JH, Bechly K, Chapman BA, Burt MJ, Barclay ML, Gearry RB, Stedman CA (2010). “Population-based epidemiology study of autoimmune hepatitis: a disease of older women?”. J. Gastroenterol. Hepatol. 25 (10): 1681–6. doi:10.1111/j.1440-1746.2010.06384.x. PMID 20880179.
↑ Primo J, Maroto N, Martínez M, Antón MD, Zaragoza A, Giner R, Devesa F, Merino C, del Olmo JA (2009). “Incidence of adult form of autoimmune hepatitis in Valencia (Spain)”. Acta Gastroenterol. Belg. 72 (4): 402–6. PMID 20163033.
↑ Grønbæk L, Vilstrup H, Jepsen P (2014). “Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study”. J. Hepatol. 60 (3): 612–7. doi:10.1016/j.jhep.2013.10.020. PMID 24326217.
↑ Feld JJ, Heathcote EJ (2003). “Epidemiology of autoimmune liver disease”. J. Gastroenterol. Hepatol. 18 (10): 1118–28. PMID 12974897.
↑ Primo J, Merino C, Fernández J, Molés JR, Llorca P, Hinojosa J (2004). “[Incidence and prevalence of autoimmune hepatitis in the area of the Hospital de Sagunto (Spain)]”. Gastroenterol Hepatol (in Spanish; Castilian). 27 (4): 239–43. PMID 15056409.
↑ Czaja AJ, dos Santos RM, Porto A, Santrach PJ, Moore SB (1998). “Immune phenotype of chronic liver disease”. Dig. Dis. Sci. 43 (9): 2149–55. PMID 9753285.
↑ Al-Chalabi T, Underhill JA, Portmann BC, McFarlane IG, Heneghan MA (2008). “Impact of gender on the long-term outcome and survival of patients with autoimmune hepatitis”. J. Hepatol. 48 (1): 140–7. doi:10.1016/j.jhep.2007.08.013. PMID 18023911.

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[pmid18609163-1] Werner M, Prytz H, Ohlsson B, Almer S, Björnsson E, Bergquist A, Wallerstedt S, Sandberg-Gertzén H, Hultcrantz R, Sangfelt P, Weiland O, Danielsson A (2008). “Epidemiology and the initial presentation of autoimmune hepatitis in Sweden: a nationwide study”. Scand. J. Gastroenterol. 43 (10): 1232–40. doi:10.1080/00365520802130183. PMID 18609163.

[pmid9489916-2] Boberg KM, Aadland E, Jahnsen J, Raknerud N, Stiris M, Bell H (1998). “Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population”. Scand. J. Gastroenterol. 33 (1): 99–103. PMID 9489916.

[pmid20880179-3] Ngu JH, Bechly K, Chapman BA, Burt MJ, Barclay ML, Gearry RB, Stedman CA (2010). “Population-based epidemiology study of autoimmune hepatitis: a disease of older women?”. J. Gastroenterol. Hepatol. 25 (10): 1681–6. doi:10.1111/j.1440-1746.2010.06384.x. PMID 20880179.

[pmid20163033-4] Primo J, Maroto N, Martínez M, Antón MD, Zaragoza A, Giner R, Devesa F, Merino C, del Olmo JA (2009). “Incidence of adult form of autoimmune hepatitis in Valencia (Spain)”. Acta Gastroenterol. Belg. 72 (4): 402–6. PMID 20163033.

[pmid24326217-5] Grønbæk L, Vilstrup H, Jepsen P (2014). “Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study”. J. Hepatol. 60 (3): 612–7. doi:10.1016/j.jhep.2013.10.020. PMID 24326217.

[pmid12974897-6] Feld JJ, Heathcote EJ (2003). “Epidemiology of autoimmune liver disease”. J. Gastroenterol. Hepatol. 18 (10): 1118–28. PMID 12974897.

[pmid15056409-7] Primo J, Merino C, Fernández J, Molés JR, Llorca P, Hinojosa J (2004). “[Incidence and prevalence of autoimmune hepatitis in the area of the Hospital de Sagunto (Spain)]”. Gastroenterol Hepatol (in Spanish; Castilian). 27 (4): 239–43. PMID 15056409.

[pmid9753285-8] Czaja AJ, dos Santos RM, Porto A, Santrach PJ, Moore SB (1998). “Immune phenotype of chronic liver disease”. Dig. Dis. Sci. 43 (9): 2149–55. PMID 9753285.

[pmid18023911-9] Al-Chalabi T, Underhill JA, Portmann BC, McFarlane IG, Heneghan MA (2008). “Impact of gender on the long-term outcome and survival of patients with autoimmune hepatitis”. J. Hepatol. 48 (1): 140–7. doi:10.1016/j.jhep.2007.08.013. PMID 18023911.

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