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Autoimmune hepatitis: a review

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Abstract

Autoimmune hepatitis (AIH) is an inflammatory liver disease that predominantly affects females. The disease is characterized histologically by interface hepatitis, biochemically by increased aspartate and alanine aminotransferase levels, and serologically by the presence of autoantibodies and elevated levels of immunoglobulin G. AIH affects both adults and children, and is particularly aggressive in the latter group. It is a relatively rare but devastating disease, which progresses rapidly unless immunosuppressive treatment is started promptly. Treatment is often successful at inducing remission of disease, and this can lead to a normal life expectancy. However, progression to cirrhosis can and does occur in some. For those with advanced-stage disease and complications, consideration of liver transplantation is appropriate.

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References

  1. Czaja AJ, Manns MP. Advances in the diagnosis, pathogenesis, and management of autoimmune hepatitis. Gastroenterology. 2010;139(1):58–72 e4.

    Article  PubMed  CAS  Google Scholar 

  2. Krawitt EL. Autoimmune hepatitis. N Engl J Med. 2006;354(1):54–66.

    Article  PubMed  CAS  Google Scholar 

  3. Bjornsson E, Talwalkar J, Treeprasertsuk S, Kamath PS, Takahashi N, Sanderson S, et al. Drug-induced autoimmune hepatitis: clinical characteristics and prognosis. Hepatology. 2010;51(6):2040–8.

    Article  PubMed  Google Scholar 

  4. Sugimoto K, Ito T, Yamamoto N, Shiraki K. Seven cases of autoimmune hepatitis that developed after drug-induced liver injury. Hepatology. 2011;54(5):1892–3.

    Article  PubMed  Google Scholar 

  5. Efe C, Purnak T, Ozaslan E, Wahlin S. Drug-induced autoimmune hepatitis caused by anti-tumor necrosis factor alpha agents. Hepatology. 2010;52(6):2246–7.

    Article  PubMed  Google Scholar 

  6. Ramakrishna J, Johnson AR, Banner BF. Long-term minocycline use for acne in healthy adolescents can cause severe autoimmune hepatitis. J Clin Gastroenterol. 2009;43(8):787–90.

    Article  PubMed  Google Scholar 

  7. Healy J, Alexander B, Eapen C, Roberts-Thomson IC. Minocycline-induced autoimmune hepatitis. Intern Med J. 2009;39(7):487–8.

    Article  PubMed  CAS  Google Scholar 

  8. Subramaniam K, Chitturi S, Brown M, Pavli P. Infliximab-induced autoimmune hepatitis in Crohn’s disease treated with budesonide and mycophenolate. Inflamm Bowel Dis. 2011;17(11):E149–50.

    Article  PubMed  CAS  Google Scholar 

  9. Goujon C, Dahel K, Berard F, Guillot I, Gunera-Saad N, Nicolas JF. Autoimmune hepatitis in two psoriasis patients treated with infliximab. J Am Acad Dermatol. 2010;63(2):e43–4.

    Article  PubMed  Google Scholar 

  10. Adar T, Mizrahi M, Pappo O, Scheiman-Elazary A, Shibolet O. Adalimumab-induced autoimmune hepatitis. J Clin Gastroenterol. 2010;44(1):e20–2.

    Article  PubMed  Google Scholar 

  11. Doyle A, Forbes G, Kontorinis N. Autoimmune hepatitis during infliximab therapy for Crohn’s disease: a case report. J Crohns Colitis. 2011;5(3):253–5.

    Article  PubMed  Google Scholar 

  12. Goldfeld DA, Verna EC, Lefkowitch J, Swaminath A. Infliximab-induced autoimmune hepatitis with successful switch to adalimumab in a patient with Crohn’s disease: the index case. Dig Dis Sci. 2011;56(11):3386–8.

    Article  PubMed  Google Scholar 

  13. 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.

    Article  PubMed  Google Scholar 

  14. Radhakrishnan KR, Alkhouri N, Worley S, Arrigain S, Hupertz V, Kay M, et al. Autoimmune hepatitis in children—impact of cirrhosis at presentation on natural history and long-term outcome. Dig Liver Dis. 2010;42(10):724–8.

    Article  PubMed  Google Scholar 

  15. Mieli-Vergani G, Vergani D. Autoimmune hepatitis. Nat Rev Gastroenterol Hepatol. 2011;8(6):320–9.

    Article  PubMed  CAS  Google Scholar 

  16. Efe C, Purnak T, Ozaslan E. Concurrent autoimmune thyroid diseases in patients with autoimmune hepatitis. J Clin Gastroenterol. 2010;44(9):660–1.

    Article  PubMed  Google Scholar 

  17. Mirzaagha F, Azali SH, Islami F, Zamani F, Khalilipour E, Khatibian M, et al. Coeliac disease in autoimmune liver disease: a cross-sectional study and a systematic review. Dig Liver Dis. 2010;42(9):620–3.

    Article  PubMed  Google Scholar 

  18. Teufel A, Weinmann A, Kahaly GJ, Centner C, Piendl A, Worns M, et al. Concurrent autoimmune diseases in patients with autoimmune hepatitis. J Clin Gastroenterol. 2010;44(3):208–13.

    Article  PubMed  CAS  Google Scholar 

  19. Bittencourt PL, Farias AQ, Porta G, Cancado EL, Miura I, Pugliese R, et al. Frequency of concurrent autoimmune disorders in patients with autoimmune hepatitis: effect of age, gender, and genetic background. J Clin Gastroenterol. 2008;42(3):300–5.

    PubMed  Google Scholar 

  20. Colecchia A, Di Biase AR, Scaioli E, Festi D. Coeliac disease and autoimmune hepatitis: gluten-free diet can influence liver disease outcome. Dig Liver Dis. 2011;43(3):247.

    Article  PubMed  Google Scholar 

  21. Panetta F, Nobili V, Sartorelli MR, Papa RE, Ferretti F, Alterio A, et al. Celiac disease in pediatric patients with autoimmune hepatitis: etiology, diagnosis, and management. Paediatr Drugs. 2012;14(1):35–41.

    Article  PubMed  Google Scholar 

  22. Mayo MJ. Management of autoimmune hepatitis. Curr Opin Gastroenterol. 2011;27(3):224–30.

    Article  PubMed  CAS  Google Scholar 

  23. Mieli-Vergani G, Vergani D. Autoimmune hepatitis in children: what is different from adult AIH? Semin Liver Dis. 2009;29(3):297–306.

    Article  PubMed  Google Scholar 

  24. Bonder A, Retana A, Winston DM, Leung J, Kaplan MM. Prevalence of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol. 2011;9(7):609–12.

    Article  PubMed  Google Scholar 

  25. Boberg KM, Chapman RW, Hirschfield GM, Lohse AW, Manns MP, Schrumpf E. Overlap syndromes: the International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue. J Hepatol. 2011;54(2):374–85.

    Article  PubMed  Google Scholar 

  26. Gonzalez LA, Orrego M, Ramirez LA, Vasquez G. Primary biliary cirrhosis/autoimmune hepatitis overlap syndrome developing in a patient with systemic lupus erythematosus: a case report and review of the literature. Lupus. 2011;20(1):108–11.

    Article  PubMed  CAS  Google Scholar 

  27. Efe C, Purnak T, Ozaslan E. The diagnostic criteria for patients with autoimmune hepatitis and primary biliary cirrhosis overlap syndrome. Clin Gastroenterol Hepatol. 2010;8(8):734.

    Article  PubMed  Google Scholar 

  28. Neuhauser M, Bjornsson E, Treeprasertsuk S, Enders F, Silveira M, Talwalkar J, et al. Autoimmune hepatitis-PBC overlap syndrome: a simplified scoring system may assist in the diagnosis. Am J Gastroenterol. 2010;105(2):345–53.

    Article  PubMed  CAS  Google Scholar 

  29. Luth S, Kanzler S, Frenzel C, Kasper HU, Dienes HP, Schramm C, et al. Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. J Clin Gastroenterol. 2009;43(1):75–80.

    Article  PubMed  Google Scholar 

  30. Al-Chalabi T, Portmann BC, Bernal W, McFarlane IG, Heneghan MA. Autoimmune hepatitis overlap syndromes: an evaluation of treatment response, long-term outcome and survival. Aliment Pharmacol Ther. 2008;28(2):209–20.

    Article  PubMed  CAS  Google Scholar 

  31. Werner M, Wallerstedt S, Lindgren S, Almer S, Bjornsson E, Bergquist A, et al. Characteristics and long-term outcome of patients with autoimmune hepatitis related to the initial treatment response. Scand J Gastroenterol. 2010;45(4):457–67.

    Article  PubMed  Google Scholar 

  32. Lamers MM, van Oijen MG, Pronk M, Drenth JP. Treatment options for autoimmune hepatitis: a systematic review of randomized controlled trials. J Hepatol. 2010;53(1):191–8.

    Article  PubMed  CAS  Google Scholar 

  33. Czaja AJ. Rapidity of treatment response and outcome in type 1 autoimmune hepatitis. J Hepatol. 2009;51(1):161–7.

    Article  PubMed  CAS  Google Scholar 

  34. Wang Q, Qiu D, Ma X. Early normalisation of aminotransferase predicts complete biochemical remission in autoimmune hepatitis patients. Aliment Pharmacol Ther. 2011;34(1):107–9.

    Article  PubMed  Google Scholar 

  35. Czaja AJ, Carpenter HA. Histological features associated with relapse after corticosteroid withdrawal in type 1 autoimmune hepatitis. Liver Int. 2003;23(2):116–23.

    Article  PubMed  Google Scholar 

  36. Snider KR, Potter TG. Budesonide for the treatment of autoimmune hepatitis. Ann Pharmacother. 2011;45(9):1144–50.

    Article  PubMed  CAS  Google Scholar 

  37. Manns MP, Woynarowski M, Kreisel W, Lurie Y, Rust C, Zuckerman E, et al. Budesonide induces remission more effectively than prednisone in a controlled trial of patients with autoimmune hepatitis. Gastroenterology. 2010;139(4):1198–206.

    Article  PubMed  CAS  Google Scholar 

  38. Hennes EM, Oo YH, Schramm C, Denzer U, Buggisch P, Wiegard C, et al. Mycophenolate mofetil as second line therapy in autoimmune hepatitis? Am J Gastroenterol. 2008;103(12):3063–70.

    Article  PubMed  CAS  Google Scholar 

  39. Hlivko JT, Shiffman ML, Stravitz RT, Luketic VA, Sanyal AJ, Fuchs M, et al. A single center review of the use of mycophenolate mofetil in the treatment of autoimmune hepatitis. Clin Gastroenterol Hepatol. 2008;6(9):1036–40.

    Article  PubMed  CAS  Google Scholar 

  40. Fallatah HI, Akbar HO. Mycophenolate mofetil as a rescue therapy for autoimmune hepatitis patients who are not responsive to standard therapy. Expert Rev Gastroenterol Hepatol. 2011;5(4):517–22.

    Article  PubMed  CAS  Google Scholar 

  41. Schramm C, Lohse AW. Role of mycophenolate mofetil in the treatment of autoimmune hepatitis. J Hepatol. 2011;55(3):510–1.

    Article  PubMed  CAS  Google Scholar 

  42. Zachou K, Gatselis N, Papadamou G, Rigopoulou EI, Dalekos GN. Mycophenolate for the treatment of autoimmune hepatitis: prospective assessment of its efficacy and safety for induction and maintenance of remission in a large cohort of treatment-naive patients. J Hepatol. 2011;55(3):636–46.

    Article  PubMed  CAS  Google Scholar 

  43. Baven-Pronk AM, Coenraad MJ, van Buuren HR, de Man RA, van Erpecum KJ, Lamers MM, et al. The role of mycophenolate mofetil in the management of autoimmune hepatitis and overlap syndromes. Aliment Pharmacol Ther. 2011;34(3):335–43.

    Article  PubMed  CAS  Google Scholar 

  44. Garcia-Buey L, Moreno-Otero R. Mycophenolate mofetil for patients with autoimmune hepatitis and overlap syndromes. Aliment Pharmacol Ther. 2011;34(6):682–4 (author reply 4–5).

    Google Scholar 

  45. Sharzehi K, Huang MA, Schreibman IR, Brown KA. Mycophenolate mofetil for the treatment of autoimmune hepatitis in patients refractory or intolerant to conventional therapy. Can J Gastroenterol. 2010;24(10):588–92.

    PubMed  Google Scholar 

  46. Manns MP, Czaja AJ, Gorham JD, Krawitt EL, Mieli-Vergani G, Vergani D, et al. Diagnosis and management of autoimmune hepatitis. Hepatology. 2010;51(6):2193–213.

    Article  PubMed  CAS  Google Scholar 

  47. Czaja AJ, Freese DK. Diagnosis and treatment of autoimmune hepatitis. Hepatology. 2002;36(2):479–97.

    Article  PubMed  Google Scholar 

  48. Aggarwal N, Chopra S, Suri V, Sikka P, Dhiman RK, Chawla Y. Pregnancy outcome in women with autoimmune hepatitis. Arch Gynecol Obstet. 2011;284(1):19–23.

    Article  PubMed  Google Scholar 

  49. Schramm C, Herkel J, Beuers U, Kanzler S, Galle PR, Lohse AW. Pregnancy in autoimmune hepatitis: outcome and risk factors. Am J Gastroenterol. 2006;101(3):556–60.

    Article  PubMed  CAS  Google Scholar 

  50. Park SZ, Nagorney DM, Czaja AJ. Hepatocellular carcinoma in autoimmune hepatitis. Dig Dis Sci. 2000;45(10):1944–8.

    Article  PubMed  CAS  Google Scholar 

  51. Tripathi D, Neuberger J. Autoimmune hepatitis and liver transplantation: indications, results, and management of recurrent disease. Semin Liver Dis. 2009;29(3):286–96.

    Article  PubMed  Google Scholar 

  52. Martin SR, Alvarez F, Anand R, Song C, Yin W. Outcomes in children who underwent transplantation for autoimmune hepatitis. Liver Transpl. 2011;17(4):393–401.

    Article  PubMed  Google Scholar 

  53. Schramm C, Bubenheim M, Adam R, Karam V, Buckels J, O’Grady JG, et al. Primary liver transplantation for autoimmune hepatitis: a comparative analysis of the European Liver Transplant Registry. Liver Transpl. 2010;16(4):461–9.

    PubMed  Google Scholar 

  54. Chai PF, Lee WS, Brown RM, McPartland JL, Foster K, McKiernan PJ, et al. Childhood autoimmune liver disease: indications and outcome of liver transplantation. J Pediatr Gastroenterol Nutr. 2010;50(3):295–302.

    Article  PubMed  CAS  Google Scholar 

  55. Duclos-Vallee JC, Sebagh M. Recurrence of autoimmune disease, primary sclerosing cholangitis, primary biliary cirrhosis, and autoimmune hepatitis after liver transplantation. Liver Transpl. 2009;15(Suppl 2):S25–34.

    Article  PubMed  Google Scholar 

  56. Campsen J, Zimmerman MA, Trotter JF, Wachs M, Bak T, Steinberg T, et al. Liver transplantation for autoimmune hepatitis and the success of aggressive corticosteroid withdrawal. Liver Transpl. 2008;14(9):1281–6.

    Article  PubMed  Google Scholar 

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Correspondence to Andrea A. Gossard.

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Gossard, A.A., Lindor, K.D. Autoimmune hepatitis: a review. J Gastroenterol 47, 498–503 (2012). https://doi.org/10.1007/s00535-012-0586-z

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