Skip to main content
SpringerLink
Log in

Unique Aspects of Autoimmune Hepatitis in Children

  • Chapter
Liver Immunology

  • 1774 Accesses

Abstract

Autoimmune hepatitis (AIH) in childhood is an inflammatory liver disease characterized histologically by a dense portal tract mononuclear cell infiltrate and serologically by the presence of non-organ- and liver-specific autoantibodies and increased levels of IgG, in the absence of a known etiology. AIH usually responds to immunosuppressive treatment, which should be instituted as soon as a diagnosis is made. In pediatrics, as well as in young adults, AIH often presents acutely and has a more aggressive course than in older patients. In children there are two liver disorders in which the liver damage is likely to arise from an autoimmune attack: classical AIH and AIH/sclerosing cholangitis overlap syndrome (ASC). A possible autoimmune pathogenesis has also been postulated for the so-called post liver transplant de novo AIH, a condition originally described in children and later confirmed in adults.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 199.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Gregorio GV, Portmann B, Reid F, et al. Autoimmune hepatitis in childhood: a 20-year experience. Hepatology 1997; 25:541–547.

    Article  PubMed  CAS  Google Scholar 

  2. Ahonen P, Myllarniemi S, Sipila I, Perheentupa J. Clinical variation of autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) in a series of 68 patients. N Engl J Med 1990; 322: 1829–1836.

    Article  PubMed  CAS  Google Scholar 

  3. Johnson PJ, McFarlane IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology 1993; 18:998–1005.

    Article  PubMed  CAS  Google Scholar 

  4. Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol 1999; 31:929–938.

    Article  PubMed  CAS  Google Scholar 

  5. Vergani D, Alvarez F, Bianchi FB, et al. Liver autoimmune serology: a consensus statement from the committee for autoimmune serology of the International Autoimmune Hepatitis Group. J Hepatol 2004; 41:677–683.

    Article  PubMed  Google Scholar 

  6. Bottazzo GF, Florin-Christensen A, Fairfax A, Swana G, Doniach D, GroeschelStewart U. Classification of smooth muscle autoantibodies detected by immunofluorescence. J Clin Pathol 1976; 29: 403–410.

    Article  PubMed  CAS  Google Scholar 

  7. Muratori P, Muratori L, Agostinelli D, et al. Smooth muscle antibodies and type 1 autoimmune hepatitis. Autoimmunity 2002; 35:497–500.

    Article  PubMed  CAS  Google Scholar 

  8. Gregorio GV, Portmann B, Mowat AP, Vergani D, Mieli-Vergani G. A 12-yearold girl with antimitochondrial antibody-positive autoimmune hepatitis. J Hepatol 1997; 27:751–754.

    Article  PubMed  CAS  Google Scholar 

  9. Lapierre P, Hajoui O, Homberg JC, Alvarez F. Formiminotransferase cyclodeaminase is an organ-specific autoantigen recognized by sera of patients with autoimmune hepatitis. Gastroenterology 1999; 116: 643–649.

    Article  PubMed  CAS  Google Scholar 

  10. Manns M, Gerken G, Kyriatsoulis A, Staritz M, Meyer zum Buschenfelde KH. Characterisation of a new subgroup of autoimmune chronic active hepatitis by autoantibodies against a soluble liver antigen. Lancet 1987; 1:292–294.

    Article  PubMed  CAS  Google Scholar 

  11. Ma Y, Okamoto M, Thomas MG, et al. Antibodies to conformational epitopes of soluble liver antigen define a severe form of autoimmune liver disease. Hepatology 2002; 35:658–664.

    Article  PubMed  CAS  Google Scholar 

  12. Donaldson PT. Genetics in autoimmune hepatitis. Semin Liver Dis 2002; 22:353–364.

    Article  PubMed  CAS  Google Scholar 

  13. Ma Y, Bogdanos DP, Hussain MJ, et al. Polyclonal T-cell responses to cytochrome P450IID6 are associated with disease activity in autoimmune hepatitis type-2 Gastroenterology 2006; 130:868–882.

    CAS  Google Scholar 

  14. Fainboim L, Canero Velasco MC, Marcos CY, et al. Protracted, but not acute, hepatitis A virus infection is strongly associated with HLA-DRB*1301, a marker for pediatric autoimmune hepatitis. Hepatology 2001; 33:1512–1517.

    Article  PubMed  CAS  Google Scholar 

  15. Pando M, Larriba J, Fernandez GC, et al. Pediatric and adult forms of type I autoimmune hepatitis in Argentina: evidence for differential genetic predisposition. Hepatology 1999; 30:1374–1380.

    Article  PubMed  CAS  Google Scholar 

  16. Vergani D, Wells L, Larcher VF, et al. Genetically determined low C4: a predisposing factor to autoimmune chronic active hepatitis. Lancet 1985; 2:294–298.

    Article  PubMed  CAS  Google Scholar 

  17. Gregorio GV, Portmann B, Karani J, et al. Autoimmune hepatitis/ sclerosing cholangitis overlap syndrome in childhood: a 16-year prospective study. Hepatology 2001; 33:544–553.

    Article  PubMed  CAS  Google Scholar 

  18. Gregorio GV, Jones H, Choudhuri K, et al. Autoantibody prevalence in chronic hepatitis B virus infection: effect in interferon alfa. Hepatology 1996; 24:520–523.

    Article  PubMed  CAS  Google Scholar 

  19. Gregorio GV, Pensati P, Iorio R, Vegnente A, Mieli-Vergani G, Vergani D. Autoantibody prevalence in children with liver disease due to chronic hepatitis C virus (HCV) infection. Clin Exp Immunol 1998; 112:471–476.

    Article  PubMed  CAS  Google Scholar 

  20. Dhawan A, Taylor RM, Cheeseman P, De Silva P, Katsiyiannakis L, MieliVergani G. Wilson’s disease in children: 37-year experience and revised King’s score for liver transplantation. Liver Transpl 2005; 11:441–448.

    Article  PubMed  Google Scholar 

  21. Liston A, Lesage S, Gray DH, Boyd RL, Goodnow CC. Genetic lesions in T-cell tolerance and thresholds for autoimmunity. Immunol Rev 2005; 204:87–101.

    Article  PubMed  CAS  Google Scholar 

  22. Simmonds MJ, Gough SC. Genetic insights into disease mechanisms of autoimmunity. Br Med Bull 2004; 71:93–113.

    Article  PubMed  CAS  Google Scholar 

  23. Mieli-Vergani G, Vergani D. Autoimmune hepatitis in children. Clin Liver Dis 2002; 6:623–634.

    Article  PubMed  Google Scholar 

  24. Rumbo C, Emerick KM, Emre S, Shneider BL. Azathioprine metabolite measurements in the treatment of autoimmune hepatitis in pediatric patients: a preliminary report. J Pediatr Gastroenterol Nutr 2002; 35:391–398.

    Article  PubMed  CAS  Google Scholar 

  25. Gregorio GV, McFarlane B, Bracken P, Vergani D, Mieli-Vergani G. Organ and non-organ specific autoantibody titres and IgG levels as markers of disease activity: a longitudinal study in childhood autoimmune liver disease. Autoimmunity 2002; 35:515–519.

    Article  PubMed  CAS  Google Scholar 

  26. Mieli-Vergani G, Bargiota K, Samyn M, Vergani D. Therapeutic aspects of autoimmune liver disease in children. In: Dienes HP, Leuschner U, Lohse AW, Manns MP, eds. Autoimmune Liver Diseases— Falk Symposium. Dordrecht: Springer; 2005: 278–282.

    Chapter  Google Scholar 

  27. Johnson PJ, McFarlane IG, Williams R. Azathioprine for long-term maintenance of remission in autoimmune hepatitis. N Engl J Med 1995; 333:958–963.

    Article  PubMed  CAS  Google Scholar 

  28. Alvarez F, Ciocca M, Canero-Velasco C, et al. Short-term cyclosporine induces a remission of autoimmune hepatitis in children. J Hepatol 1999; 30:222–227.

    Article  PubMed  CAS  Google Scholar 

  29. Underhill J, Liaskos C, Bogdanos DP, et al. Juvenile autoimmune sclerosing cholangitis is associated with possession of HLA DRB 1*13. J Hepatol 2006; 44:S241.

    Article  Google Scholar 

  30. Lindor KD. Ursodiol for primary sclerosing cholangitis. Mayo Primary Sclerosing Cholangitis-Ursodeoxycholic Acid Study Group. N Engl J Med 1997; 336:691–695.

    Article  PubMed  CAS  Google Scholar 

  31. Mitchell SA, Bansi DS, Hunt N, Von Bergmann K, Fleming KA, Chapman RW. A preliminary trial of high-dose ursodeoxycholic acid in primary sclerosing cholangitis. Gastroenterology 2001; 121: 900–907.

    Article  PubMed  CAS  Google Scholar 

  32. Kerkar N, Hadzic N, Davies ET, et al. De-novo autoimmune hepatitis after liver transplantation. Lancet 1998; 351:409–413.

    Article  PubMed  CAS  Google Scholar 

  33. Vergani D, Mieli-Vergani G. Autoimmunity after liver transplantation. Hepatology 2002; 36:271–276.

    Article  PubMed  CAS  Google Scholar 

  34. Mieli-Vergani G, Vergani D. De novo autoimmune hepatitis after liver transplantation. J Hepatol 2004; 40:3–7.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Institute of Liver Studies, King’s College London School of Medicine at King’s College Hospital, London, UK

    Giorgina Mieli-Vergani MD, PhD

  2. Departments of Medicine and Surgery, Baylor Liver Health, Baylor College of Medicine, Houston, TX

    Diego Vergani MD, FACP

Authors
  1. Giorgina Mieli-Vergani MD, PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Diego Vergani MD, FACP
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, Davis, CA

    M. Eric Gershwin MD, FACP

  2. Departments of Medicine and Surgery Baylor Liver Health, Baylor College of Medicine, Houston, TX

    John M. Vierling MD, FACP

  3. Department of Gastroenterology, Hepatology, and Endocrinology, Medical School of Hannover, Hannover, Germany

    Michael P. Manns MD

Rights and permissions

Reprints and permissions

Copyright information

© 2007 Humana Press Inc., Totowa, NJ

About this chapter

Cite this chapter

Mieli-Vergani, G., Vergani, D. (2007). Unique Aspects of Autoimmune Hepatitis in Children. In: Gershwin, M.E., Vierling, J.M., Manns, M.P. (eds) Liver Immunology. Humana Press. https://doi.org/10.1007/978-1-59745-518-3_22

Download citation

  • DOI: https://doi.org/10.1007/978-1-59745-518-3_22

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-818-8

  • Online ISBN: 978-1-59745-518-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation