Skip to main content

Clinical Presentation

  • Chapter
  • First Online:
Book cover Autoimmune Hepatitis

Part of the book series: Clinical Gastroenterology ((CG))

  • 1118 Accesses

Abstract

Waldenstrom was the first to describe what later became recognized as Autoimmune Hepatitis (AIH). No particular ethnicity precludes the development of AIH although its presentation may vary across the world. AIH may present at any age, from infancy through to the elderly. Although more common in women this disease must nevertheless be considered in the differential diagnosis of “hepatitis” in men. When AIH was first recognized as a specific entity it was assumed that the disease was always symptomatic. Manifestations of advanced liver disease were usual at first presentation. The recent introduction of routine screening blood tests indicates that some individuals may have “asymptomatic” AIH. At the other end of the spectrum, the dramatic improvement in the understanding and management of Fulminant Hepatic Failure (FHF) has allowed the recognition of AIH as one of its many causes. Liver transplant saves the lives of most of those who need it, although recurrent AIH in the new liver may develop.

It is important to recognize the change in demography over the last 40 years as the patients who were entered into any one of the three “classic” randomized control trials of immunosuppressive therapy for AIH in the late 1960s do not necessarily represent the patient population seen in the physician’s office or emergency room today. Thus, it is wrong to assume that the therapies shown to be lifesaving in those with advanced liver disease benefit all those given a diagnosis of AIH today, particularly those at the two extremes eluded to above.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. WJ Leber. Glutproteine und nahrungselweiss Stofwechs. Bad Kissingen. Krk. Sonderband XV. Tagung 1950;8.

    Google Scholar 

  2. Cook GC, Mulligan R, Sherlock S. Controlled prospective trial of corticosteroid therapy in active chronic Hepatitis. Q J Med. 1971;158:159.

    Google Scholar 

  3. Soloway RD, Summerskill WHJ, Baggenstoss AH, et al. Clinical, biochemical, and histological remission of severe chronic active liver disease: a controlled study of treatments and early prognosis. Gastroenterology. 1972;63:820.

    PubMed  CAS  Google Scholar 

  4. Murray-Lyon IM, Stern RB, Williams R. Controlled trial of prednisone and azathioprine in acute chronic hepatitis. Lancet. 1973;1:735.

    Article  PubMed  CAS  Google Scholar 

  5. Vento S, Guella L, Mirandola F, et al. Epstein-barr virus as a trigger for autoimmune hepatitis in susceptible individuals. Lancet. 1995;346:608.

    Article  PubMed  CAS  Google Scholar 

  6. Vento S, Cainelli F, Ferraro T, et al. Autoimmune hepatitis type 1 after measles. Am J Gastroenterol. 1996;91:2618.

    PubMed  CAS  Google Scholar 

  7. Ferrari R, Pappas G, Agostinelli D, Muratori P, Muratori L, Lenzi M, et al. Type 1 autoimmune hepatitis: patterns of clinical presentation and differential diagnosis of the ‘Acute’ type. Q J Med. 2004;97:407–12.

    Article  CAS  Google Scholar 

  8. Miyake Y, Iwasaki Y, Kobashi H, Yasunaka T, Ikeda F, Takaki A, et al. Autoimmune hepatitis with acute presentation in Japan. Dig Liver Dis. 2010;42(1):51–4.

    Article  PubMed  CAS  Google Scholar 

  9. Alvarez F, Berg FB, Bianchi L, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999;31:929.

    Article  PubMed  CAS  Google Scholar 

  10. Hennes EM, Zeniya M, Czaja AJ, Parés A, Dalekos GN, Krawitt EL, et al. International Autoimmune Hepatitis Group. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology. 2008;48:169–76.

    Article  PubMed  Google Scholar 

  11. Yeoman AD, Westbrook RH, Al-Chalabi T, Carey I, Heaton ND, Portmann BC, et al. Diagnostic value and utility of the simplified International Autoimmune Hepatitis Group (IAIHG) criteria in acute and chronic liver disease. Hepatology. 2009;50:538–45.

    Article  PubMed  Google Scholar 

  12. 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;42:53–62.

    Article  PubMed  Google Scholar 

  13. Czaja AJ, Carpenter HA, Santrach PJ, Moore SB, Homburger HA. The nature and prognosis of severe cryptogenic chronic active hepatitis. Gastroenterology. 1993;104:1755–61.

    PubMed  CAS  Google Scholar 

  14. Hiramatsu A, Takahashi S, Aikata H, Azakami T, Katamura Y, Kawaoka T, et al. Etiology and outcome of acute liver failure: retrospective analysis of 50 patients treated at a single center. J Gastroenterol Hepatol. 2008;23:1216–22.

    Article  PubMed  Google Scholar 

  15. Santos RG, Alissa F, Reyes J, Teot L, Ameen N. Fulminant hepatic failure: Wilson’s disease or autoimmune hepatitis? Implications for transplantation. Pediatr Transplant. 2005;9:112–6.

    Article  PubMed  CAS  Google Scholar 

  16. Kessler WR, Cummings OW, Eckert G, Chalasani N, Lumeng L, Kwo PY. Fulminant hepatic failure as the initial presentation of acute autoimmune hepatitis. Clin Gastroenterol Hepatol. 2004;2:625–31.

    Article  PubMed  Google Scholar 

  17. Núñez-Martínez O, De la Cruz G, Salcedo M, Molina J, De Diego A, Ripoll C, et al. Liver transplantation for autoimmune hepatitis: fulminant versus chronic hepatitis presentation. Transplant Proc. 2003;35:1857–8.

    Article  PubMed  Google Scholar 

  18. 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 

  19. Montano-Loza AJ, Mason AL, Ma M, Bastiampillai RJ, Bain VG, Tandon P. Risk factors for recurrence of autoimmune hepatitis after liver transplantation. Liver Transpl. 2009;15:1254–61.

    Article  PubMed  Google Scholar 

  20. Gautam M, Cheruvattath R, Balan V. Recurrence of autoimmune liver disease after liver transplantation: a systematic review. Liver Transpl. 2006;12:1813–24.

    Article  PubMed  Google Scholar 

  21. Gregorio GV, Portmann B, Karani J, Harrison P, Donaldson PT, Vergani D, et al. Autoimmune hepatitis/sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology. 2001;33:544–53.

    Article  PubMed  CAS  Google Scholar 

  22. Wilschanski M, Chait P, Wade JA, Davis L, Corey M, St Louis P, et al. Primary sclerosing cholangitis in 32 children: clinical, laboratory, and radiographic features, with survival analysis. Hepatology. 1995;22:1415–22.

    PubMed  CAS  Google Scholar 

  23. Abdalian R, Dhar P, Jhaveri K, Haider M, Guindi M, Heathcote EJ. Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: evaluating the role of routine magnetic resonance imaging. Hepatology. 2008;47(3):949–57.

    Article  PubMed  CAS  Google Scholar 

  24. Lewin M, Vilgrain V, Ozenne V, Lemoine M, Wendum D, Paradis V, et al. Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis: a prospective magnetic resonance imaging and histological study. Hepatology. 2009;50(2):528–37.

    Article  PubMed  Google Scholar 

  25. Floreani A, Rizzotto ER, Ferrara F, Carderi I, Caroli D, Blasone L, et al. Clinical course and outcome of autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome. Am J Gastroenterol. 2005;100(7):1516–22.

    Article  PubMed  Google Scholar 

  26. 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 

  27. Abdo AA, Bain VG, Kichian K, Lee SS. Evolution of autoimmune hepatitis to primary sclerosing cholangitis: a sequential syndrome. Hepatology. 2002;36(6):1393–9.

    PubMed  Google Scholar 

  28. Colombato LA, Alvarez F, Côté J, Huet PM. Autoimmune cholangiopathy: the result of consecutive primary biliary cirrhosis and autoimmune hepatitis? Gastroenterology. 1994;107(6):1839–43.

    PubMed  CAS  Google Scholar 

  29. Chazouillères O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: clinical features and response to therapy. Hepatology. 1998;28(2):296–301.

    Article  PubMed  Google Scholar 

  30. Poupon R, Chazouilleres O, Corpechot C, Chrétien Y. Development of autoimmune hepatitis in patients with typical primary biliary cirrhosis. Hepatology. 2006;44(1):85–90.

    Article  PubMed  Google Scholar 

  31. Bhat M, Guindi M, Heathcote EJ, Hirschfield GM. Transient development of anti-mitochondrial antibodies accompanies autoimmune hepatitis-sclerosing cholangitis overlap. Gut. 2009;58(1):152–3.

    Article  PubMed  CAS  Google Scholar 

  32. Leung PS, Rossaro L, Davis PA, Park O, Tanaka A, Kikuchi K, et al. Antimitochondrial antibodies in acute liver failure: implications for primary biliary cirrhosis. Hepatology. 2007;46(5):1436–42.

    Article  PubMed  CAS  Google Scholar 

  33. Silveira MG, Talwalkar JA, Angulo P, Lindor KD. Overlap of autoimmune hepatitis and primary biliary cirrhosis: long-term outcomes. Am J Gastroenterol. 2007;102(6):1244–50.

    Article  PubMed  Google Scholar 

  34. O’Brien C, Joshi S, Feld JJ, Guindi M, Dienes HP, Heathcote EJ. Long-term follow-up of antimitochondrial antibody-positive autoimmune hepatitis. Hepatology. 2008;48(2):550–6.

    Article  PubMed  Google Scholar 

  35. Verma S, Torbenson M, Thuluvath PJ. The Impact of ethnicity on the natural history of autoimmune hepatitis. Hepatology. 2007;46(6):1828–35.

    Article  PubMed  Google Scholar 

  36. 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):3390–4.

    Article  PubMed  CAS  Google Scholar 

  37. Zolfino T, Heneghan MA, Norris S, et al. Characteristics of autoimmune hepatitis in patients who are not of European Caucasoid ethnic origin. Gut. 2002;50:713–7.

    Article  PubMed  CAS  Google Scholar 

  38. D’Souza R, Sinnott P, Glynn MJ, Sabin CA, Foster GR. An unusual form of autoimmune hepatitis in young Somalian men. Liver Int. 2005;25:325–30.

    Article  PubMed  Google Scholar 

  39. Mudawi HMY, Yousif BA. Fulminant hepatic failure in an African setting: etiology, clinical course, and predictors of mortality. Dig Dis Sci. 2007;52:3266–9.

    Article  PubMed  CAS  Google Scholar 

  40. Czaja AJ, Souto EO, Bittencourt PL, Cancado EL, Porta G, Goldberg AC, et al. Clinical distinctions and pathogenic implications of type 1 autoimmune hepatitis in Brazil and the United States. J Hepatol. 2002;37(3):302–8.

    Article  PubMed  Google Scholar 

  41. Hurlburt KJ, McMahon BJ, Deubner H, Hsu-Trawinski B, Williams JL, Kowdley KV. Prevalence of autoimmune liver disease in Alaska natives. Am J Gastroenterol. 2002;97(9):2402–7.

    Article  PubMed  Google Scholar 

  42. Werner M, Prytz H, Ohlsson B, Almer S, Björnsson E, Bergquist A, et al. Epidemiology and the initial presentation of autoimmune hepatitis in Sweden: a nationwide study. Scand J Gastroenterol. 2008;43(10):1232–40.

    Article  PubMed  Google Scholar 

  43. Minuk GY, Liu S, Kaita K, Wong S, Renner E, Rempel J, et al. Autoimmune hepatitis in a North American Aboriginal/First Nations population. Can J Gastroenterol. 2008;22(10):829–34.

    PubMed  CAS  Google Scholar 

  44. Gupta R, Agarwal SR, Jain M, Malhotra V, Sarin SK. Autoimmune hepatitis in the Indian subcontinent: 7 years experience. J Gastroenterol Hepatol. 2001;16:1144–8.

    Article  PubMed  CAS  Google Scholar 

  45. Choudhuri G, Somani SK, Baba CS, Alexander G. Autoimmune hepatitis in India: profile of an uncommon disease. BMC Gastroenterol. 2005;5:1–27.

    Article  Google Scholar 

  46. Huang HC, Huang YS, Wu JC, Tsay SH, Huo TI, Wang YJ, et al. Characteristics of autoimmune hepatitis in Taiwan: the 11 years’ experience of a medical center. Chinese Med J. 2002;65:563–9.

    Google Scholar 

  47. Czaja AJ, Carpenter HA. Distinctive clinical phenotype and treatment outcome of type 1 autoimmune hepatitis in the elderly. Hepatology. 2006;43(3):532–8.

    Article  PubMed  Google Scholar 

  48. Granito A, Muratori L, Pappas G, Muratori P, Ferri S, Cassani F, et al. Clinical features of type 1 autoimmune hepatitis in elderly Italian patients. Ailment Pharmacol Ther. 2005;21:1273–7.

    Article  CAS  Google Scholar 

  49. Miyake Y, Iwasaki Y, Kobashi H, Yasunaka T, Ikeda F, Takaki A, et al. Clinical features of type 1 autoimmune hepatitis in adolescence and early adulthood. Hepatol Res. 2009;39:766–71.

    Article  PubMed  CAS  Google Scholar 

  50. Miyake Y, Iwasaki Y, Takaki A, Kobashi H, Sakaguchi K, Shiratori Y. Clinical features of Japanese elderly patients with type 1 autoimmune hepatitis. Intern Med. 2007;46:1945–9. doi:10.2169/internalmedicine.46.0420.

    Article  PubMed  Google Scholar 

  51. Al-Chalabi T, Underhill JA, Portmann BC, McFarlane IG, Heneghan MA. Impact of gender on the long-term outcome and survival of patients with autoimmune hepatitis. J Hepatol. 2008;48:140–7.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to E. Jenny Heathcote .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2012 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Heathcote, E.J. (2012). Clinical Presentation. In: Hirschfield, G., Heathcote, E. (eds) Autoimmune Hepatitis. Clinical Gastroenterology. Springer, New York, NY. https://doi.org/10.1007/978-1-60761-569-9_3

Download citation

  • DOI: https://doi.org/10.1007/978-1-60761-569-9_3

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-60761-568-2

  • Online ISBN: 978-1-60761-569-9

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics