Diagnosis and presenting features of autoimmune hepatitis at a central referral hospital in South Africa


  • Y Maharaj Division of Internal Medicine, College of Health Sciences, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa https://orcid.org/0009-0006-6194-7419
  • V G Naidoo Department of Gastroenterology, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa; Gastrointestinal Cancer Research Group, Nelson R Mandela School of Medicine, University of KwaZulu-Natal, Durban, South Africa https://orcid.org/0000-0003-3774-9608




South Africa, autoimmune hepatitis, cirrhosis, HIV



Autoimmune hepatitis (AIH) has scarcely been reported on in patients of black African descent. Similarly, few studies have focused on the relationship between AIH and Human-Immunodeficiency Virus (HIV) infection.


We aim to describe the presenting features of AIH from a single referral centre in a Sub-Sahara African setting. We also compare the presenting features of HIV-infected and HIV-uninfected patients.


This study was a retrospective chart review. Patients were included if they fulfilled criteria for the International AIH Group simplified score for probable or definite AIH, were 18 years or older at inclusion, and attended the adult Gastroenterology clinic at Inkosi Albert Luthuli Central Hospital (IALCH) for the period 1/1/2015 to 31/12/2020 on at least 2 occasions.


Forty cases were included, of which 33 (82.5%) were female and 33 (82.5%) were black African. Median age at diagnosis was 26 years. A diagnosis of a coexistent autoimmune disease was made in 22.5% of patients, with Systemic Lupus Erythematosus (SLE) being the most common (12.5%). Sixteen patients were HIV-infected, all of whom were female (p =0.03), with a significantly older age of disease onset as compared to their HIV-uninfected counterparts (median age 38 vs 17.5 years, p <0.001).


AIH is a disease most commonly affecting young females. Female sex and older age of onset is associated with AIH in HIV-infected individuals.


Wang Q, Yang F, Miao Q, Krawitt EL, Gershwin ME, Ma X. The clinical phenotypes of autoimmune hepatitis: A comprehensive review. J Autoimmun. 2016;66:98-107. http://dx.doi.org/10.1016/j.jaut.2015.10.006

Czaja AJ, Donaldson PT. Gender effects and synergisms with histocompatibility leukocyte antigens in type 1 autoimmune hepatitis. Am J Gastroenterol. 2002;97(8):2051-2057. http://dx.doi.org/10.1111/j.1572-0241.2002.05921.x

Krawitt EL. Autoimmune hepatitis. N Engl J Med. 2006;354(1):54-66. http://dx.doi.org/10.1056/nejmra050408

van Gerven NM, de Boer YS, Mulder CJ, van Nieuwkerk CM, Bouma G. Autoimmune hepatitis. World J Gastroenterol. 2016;22(19):4651-4661. http://dx.doi.org/10.3748/wjg.v22.i19.4651

Hennes EM, Zeniya M, Czaja AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 2008;48(1):169-176. http://dx.doi.org/10.1002/hep.22322

Jemilohun A, Ola T. Autoimmune liver diseases in native Black Africans: extremely rare or inadequately investigated diseases? Journal of Advances in Medicine and Medical Research. 2017;24(7):1-14. http://dx.doi.org/10.9734/jammr/2017/36681

Sonderup MW, Wainwright H, Hall P, Hairwadzi H, Spearman CW. A clinicopathological cohort study of liver pathology in 301 patients with human immunodeficiency virus/acquired immune deficiency syndrome. Hepatology. 2015;61(5):1721-1729. http://dx.doi.org/10.1002/hep.27710

Parekh S, Spiritos Z, Reynolds P, Parekh S, Perricone A, Quigley B. HIV and autoimmune hepatitis: a case series and literature review. J Biomedical Sci. 2017;6(2):1-4. http://dx.doi.org/10.4172/2254-609x.100057

Enomoto H, Nishiguchi S. Similarities and Differences in Autoimmune Hepatitis Epidemiology between East and West: Autoimmune Hepatitis in East Asia, Southeast Asia, and South Asia. Inflamm Intest Dis. 2017;1(4):150-158. http://dx.doi.org/10.1159/000454879

de Boer YS, Gerussi A, van den Brand FF, et al. Association Between Black Race and Presentation and Liver-Related Outcomes of Patients With Autoimmune Hepatitis. Clin Gastroenterol Hepatol. 2019;17(8):1616-1624. http://dx.doi.org/10.1016/j.cgh.2018.11.028

Tunio NA, Mansoor E, Sheriff MZ, Cooper GS, Sclair SN, Cohen SM. Epidemiology of Autoimmune Hepatitis (AIH) in the United States Between 2014 and 2019: A Population-based National Study. J Clin Gastroenterol. 2021;55(10):903-910. http://dx.doi.org/10.1097/mcg.0000000000001449

Yassin S, De Lacy R, Pillay K, Goddard E. Characteristics and Outcomes of Autoimmune Hepatitis from a Tertiary Paediatric Centre, Cape Town, South Africa. J Trop Pediatr. 2020;66(4):448-457. http://dx.doi.org/10.1093/tropej/fmz088

Abe M, Mashiba T, Zeniya M, Yamamoto K, Onji M, Tsubouchi H. Present status of autoimmune hepatitis in Japan: a nationwide survey. J Gastroenterol. 2011;46(9):1136-1141. http://dx.doi.org/10.1007/s00535-011-0421-y

Kim BH, Kim YJ, Jeong SH, et al. Clinical features of autoimmune hepatitis and comparison of two diagnostic criteria in Korea: a nationwide, multicenter study. J Gastroenterol Hepatol. 2013;28(1):128-134. http://dx.doi.org/10.1111/j.1440-1746.2012.07292.x

Koay LB, Lin CY, Tsai SL, et al. Type 1 autoimmune hepatitis in Taiwan: diagnosis using the revised criteria of the International Autoimmune Hepatitis Group. Dig Dis Sci. 2006;51(11):1978-1984. http://dx.doi.org/10.1007/s10620-005-9068-y

van Gerven NM, Verwer BJ, Witte BI, et al. Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands. Scand J Gastroenterol. 2014;49(10):1245-1254. http://dx.doi.org/10.3109/00365521.2014.946083

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;60(3):612-617. http://dx.doi.org/10.1016/j.jhep.2013.10.020

Werner M, Prytz H, Ohlsson B, et al. Epidemiology and the initial presentation of autoimmune hepatitis in Sweden: a nationwide study. Scand J Gastroenterol. 2008;43(10):1232-1240. http://dx.doi.org/10.1080/00365520802130183

Muratori P, Granito A, Quarneti C, et al. Autoimmune hepatitis in Italy: the Bologna experience. J Hepatol. 2009;50(6):1210-1218. http://dx.doi.org/10.1016/j.jhep.2009.01.020

Gordon V, Adhikary R, Appleby V, et al. Diagnosis, presentation and initial severity of Autoimmune Hepatitis (AIH) in patients attending 28 hospitals in the UK. Liver Int. 2018;38(9):1686-1695. http://dx.doi.org/10.1111/liv.13724

Feld JJ, Dinh H, Arenovich T, Marcus VA, Wanless IR, Heathcote EJ. Autoimmune hepatitis: effect of symptoms and cirrhosis on natural history and outcome. Hepatology. 2005;42(1):53-62. http://dx.doi.org/10.1002/hep.20732

Hassan N, Siddiqui AR, Abbas Z, et al. Clinical Profile and HLA Typing of Autoimmune Hepatitis From Pakistan. Hepat Mon. 2013;13(12):e13598. http://dx.doi.org/10.5812/hepatmon.13598

Amarapurkar D, Dharod M, Amarapurkar A. Autoimmune hepatitis in India: single tertiary referral centre experience. Trop Gastroenterol. 2015;36(1):36-45. http://dx.doi.org/10.7869/tg.243

Lim KN, Casanova RL, Boyer TD, Bruno CJ. Autoimmune hepatitis in African Americans: presenting features and response to therapy. Am J Gastroenterol. 2001;96(12):3390-3394. http://dx.doi.org/10.1111/j.1572-0241.2001.05272.x

Johnson PJ, McFarlane IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology. 1993;18(4):998-1005. http://dx.doi.org/10.1002/hep.1840180435

Feld JJ, Heathcote EJ. Epidemiology of autoimmune liver disease. J Gastroenterol Hepatol. 2003;18(10):1118-1128. http://dx.doi.org/10.1046/j.1440-1746.2003.03165.x

Guo L, Zhou L, Zhang N, Deng B, Wang B. Extrahepatic Autoimmune Diseases in Patients with Autoimmune Liver Diseases: A Phenomenon Neglected by Gastroenterologists. Gastroenterol Res Pract. 2017;2017:1-7. http://dx.doi.org/10.1155/2017/2376231

Teufel A, Weinmann A, Kahaly GJ, et al. Concurrent autoimmune diseases in patients with autoimmune hepatitis. J Clin Gastroenterol. 2010;44(3):208-213. http://dx.doi.org/10.1097/mcg.0b013e3181c74e0d

Fogel R, Comerford M, Chilukuri P, Orman E, Chalasani N, Lammert C. Extrahepatic Autoimmune Diseases are Prevalent in Autoimmune Hepatitis Patients and Their First-Degree Relatives: Survey Study. Interact J Med Res. 2018;7(2):e18. http://dx.doi.org/10.2196/ijmr.9625

Wong GW, Yeong T, Lawrence D, Yeoman AD, Verma S, Heneghan MA. Concurrent extrahepatic autoimmunity in autoimmune hepatitis: implications for diagnosis, clinical course and long-term outcomes. Liver Int. 2017;37(3):449-457. http://dx.doi.org/10.1111/liv.13236

Muratori P, Fabbri A, Lalanne C, Lenzi M, Muratori L. Autoimmune liver disease and concomitant extrahepatic autoimmune disease. Eur J Gastroenterol Hepatol. 2015;27(10):1175-1179. http://dx.doi.org/10.1097/meg.0000000000000424

Chaiteerakij R, Sanpawat A, Avihingsanon A, Treeprasertsuk S. Autoimmune hepatitis in human immunodeficiency virus-infected patients: A case series and review of the literature. World J Gastroenterol. 2019;25(35):5388-5402. http://dx.doi.org/10.3748/wjg.v25.i35.5388

Mubder M, Azab M, Jayaraj M, et al. Autoimmune hepatitis in patients with human immunodeficiency virus infection: A systematic review of the published literature. Medicine (Baltimore). 2019;98(37):e17094. http://dx.doi.org/10.1097/md.0000000000017094

Root-Bernstein R. Antigenic complementarity between HIV and other AIDS-associated infections results in idiotype-antiidiotype antibody complexes that cross react with lymphocyte proteins. Vaccine. 2005;23(17-18):2160-2163. http://dx.doi.org/10.1016/j.vaccine.2005.01.049







How to Cite

Maharaj Y, Naidoo VG. Diagnosis and presenting features of autoimmune hepatitis at a central referral hospital in South Africa. S Afr Med J [Internet]. 2024 Apr. 18 [cited 2024 Jul. 14];114(4). Available from: https://samajournals.co.za/index.php/samj/article/view/1646

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