The Indian Journal of Pediatrics

, Volume 62, Issue 5, pp 525–531 | Cite as

Autoimmune hepatitis

  • Eve A. Roberts
Symposium: Hepatology—Part I


Autoimmune hepatitis can present as either acute or chronic disease in children. Clinical and laboratory features, including association with extrahepatic autoimmune syndromes and prompt response to immunosuppressive treatment, circulating autoantibodies and hypergammaglobulinemia, suggest an immune etiology. However, the disease mechanism remains uncertain. Different types of autoimmune hepatitis are defined on the basis of which autoantibodies are present: anti-smooth muscle (type 1), anti-liver/kidney microsomal (type 2), or anti-soluble liver antigen (type 3). Diseases which may be clinically similar to autoimmune hepatitis must be excluded before the diagnosis of autoimmune hepatitis is established: Wilson’s disease, primary sclerosing cholangitis, chronic hepatitis B or C, and drug-induced liver disease are among the most important entities. Corticosteroids alone or with azathioprine constitute the usual treatment for autoimmune hepatitis. Although some children achieve a complete remission, or even recovery, and can stop immunosuppressive treatment, others require low-dose prednisone treatment indefinitely.

Key words

Autoimmune hepatitis Autoantibodies Anti-smooth muscle antibodies Anti-liver/kidney microsomal antibodies Immunosuppressive treatment Childhood liver disease 


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  1. 1.
    Johnson PJ, McFarlane IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology 1993; 18: 998–1005.PubMedCrossRefGoogle Scholar
  2. 2.
    Manns M, Johnson EF, Griffin KJet al. Major target of liver kidney microsomal antibodies in idiopathic autoimmune heapatitis is cytochrome P450 dbl.J Clin Invest 1989; 83: 1066–1072.PubMedGoogle Scholar
  3. 3.
    Manns MP, Griffin KJ, Sullivan KF, Johnson EF. LKM-1 autoantibodies recognize a short linear sequence in P450 IID6, a cytochrome P-450 monooxygenase.J Clin Invest 1991; 88: 1370–1378.PubMedCrossRefGoogle Scholar
  4. 4.
    Michel G, Ritter A, Gerken Get al. Anti-GOR and hepatitis C virus in autoimmune liver diseases.Lancet 1992; 339: 267–269.PubMedCrossRefGoogle Scholar
  5. 5.
    Mackie FD, Peakmen M, Yun Met al. Primary and secondary liver/kidney microsomal autopatnibody response following infection with hepatitis C virus.Gastronterology 1994; 106: 1672–1675.Google Scholar
  6. 6.
    Wächter B, Kyriatsoulis A, Lohse AWet al. Characterization of liver cytokeratin as a major target antigen of antibodies.J Hepatol 1990; 11: 232–239.PubMedCrossRefGoogle Scholar
  7. 7.
    Mieli-Vergani G, Vergani D. Progress in pediatric autoimmune hepatitis.Sem Liver Dis 1994; 14: 282–288.Google Scholar
  8. 8.
    Manns MP, Krüger M. Immunogenetics of chronic liver diseases.Gastroenterology 1994; 106: 1676–1679.PubMedGoogle Scholar
  9. 9.
    Meyer zum Büschenfelde KH, Lohse AW, Manns M, Poralla T. Autoimmunity and liver disease.Hepatology 1990; 12: 354–363.PubMedCrossRefGoogle Scholar
  10. 10.
    Donaldson PT, Doherty DG, Hayllar KMet al. Susceptibility to autoimmune chronic active hepatitis: human leukocyte antigens DR4 and A1-B8-DR3 are independent risk factors.Hepatology 1991; 13: 701–706.PubMedGoogle Scholar
  11. 11.
    Vergani D, Wells L, Larcher VF,et al. Genetically determined low C4: a predisposing factor to autoimmune chronic active hepatitis.Lancet 1985; ii: 294–298.CrossRefGoogle Scholar
  12. 12.
    Scully LJ, Toze C, Sengar DPS, Goldstein R. Early-onset autoimmune hepatitis is associated with a C4A gene deletion.Gastroenterology 1993; 104: 1478–1484.PubMedGoogle Scholar
  13. 13.
    Vajro P, Hadchouel P, Hadchouel Met al. Incidence of cirrhosis in children with chronic hepatitis.J Pediatr 1990; 117: 392–396.PubMedCrossRefGoogle Scholar
  14. 14.
    Vegnente A, Larcher VF, Mowat APet al. Duration of chronic active hepatitis and the development of cirrhosis.Arch Dis Child 1984; 59: 330–335.PubMedGoogle Scholar
  15. 15.
    Maggiore G, Bernard O, Homberg JCet al. Liver disease associated with anti-liver-kidney microsome antibody in children.J Pediatr 1986; 108: 399–404.PubMedCrossRefGoogle Scholar
  16. 16.
    Homberg JC, Abuaf N, Bernard Oet al. Chronic active hepatitis associated with antiliver/kidney microsome antibody type 1: A second type of “autoimmune” hepatitis.Hepatology 1987; 7: 1333–1339.PubMedCrossRefGoogle Scholar
  17. 17.
    Gregorio GV, Portmann B, Vergani Det al. Autoimmune hepatitis of childhood: a 20 year review.Hepatology 1993; 18: 175A (abstr).CrossRefGoogle Scholar
  18. 18.
    Porta G, da Costa Gayotto LC, Alvarez F. Anti-liver-kidney microsome antibody-positive autoimmune hepatitis presenting as fulminant liver failure.J Pediatr Gastroenterol Nutrit 1990; 11: 138–140.Google Scholar
  19. 19.
    Maggiore G, Porta G, Bernard Oet al. Autoimmune hepatitis with initial presentation as acute hepatitic failure in young children.J Pediatr 1990; 116: 280–282.PubMedCrossRefGoogle Scholar
  20. 20.
    Manns M, Gerken G, Kyriatsoulis Aet al. Characterisation of a new subgroup of autoimmune chronic active hapatitis by autoantibodies against a soluble liver antigen.Lancet 1987; i: 292–294.CrossRefGoogle Scholar
  21. 21.
    Scott J, Gollan JL, Samourian S, Sherlock S. Wilson’s disease presenting as chronic active hepatitis.Gastroenterology 1978; 74: 645–651.PubMedGoogle Scholar
  22. 22.
    Schilsky ML, Scheinberg IH, Sternlieb I. Prognosis of Wilsonian chronic active hepatitis.Gastroenterology 1991; 100: 762–767.PubMedGoogle Scholar
  23. 23.
    El-Shabrawi M, Wilkinson M, Portmann Bet al. Primary sclerosing cholangitis in childhood.Gastroenterology 1987; 92: 1226–1235.PubMedGoogle Scholar
  24. 24.
    Mieli-Vergani G, Lobo-Yeo A, McFarlane BMet al. Differnt immune mechanisms lead to atuoimmunity in primary sclerosing choangitis and autoimmune chronic active haptitis of childhood.Hepatology 1989; 9: 198–203.PubMedCrossRefGoogle Scholar
  25. 25.
    Wilschanski M, Roberts E, Blendis LMet al. Primary sclerosing cholangitis in patinets <20 years old: diversity of clinical and laboratory features differing from adult disease patterns.Hepatology 1993; 18: 174A (abstr).Google Scholar
  26. 26.
    Desmet VJ, Gerber M, Hoofnagel JHet al. classification of chronic hepatitis: diagnosis, grading and staging.Hepatology 1994; 19: 1513–1520.PubMedGoogle Scholar
  27. 27.
    Cook CG, Mulligan R, Sherlock S. Controlled prospective trial of corticosteroid therapy in active chornic hepatitis.Quart J Med 1971; 40: 159–185.PubMedGoogle Scholar
  28. 28.
    Soloway RD, Summerskill WHJ, Baggenstoss AHet al. Clinical biochemical and histological remission of severe chronic active liver disease: a controlled study of treatments and early prognosis.Gastroenterology 1972; 63: 820–833.PubMedGoogle Scholar
  29. 29.
    Murray-Lyon IM, Stern RB, Williams R. Controlled trial of predisone and azathioprine in active chronic hepatitis.Lancet 1973; i: 735–737.CrossRefGoogle Scholar
  30. 30.
    Kirk AP, Jain S, Pocock, Set al. Late results of the Royal Free Hospital prospective controlled trial of prenisolone therapy in hepatitis B surface antigen negative chronic active hepatitis.Gut 1980; 21: 78–83.PubMedCrossRefGoogle Scholar
  31. 31.
    Czaja AJ, Beaver SJ, Shiels MT. Sustained remission after corticosteroid therapy of severe hepatitis B surface antigen-negative chronic active hepatitis.Gastroenterology 1987; 92: 215–219.PubMedGoogle Scholar
  32. 32.
    Hetgarty JE, Aria KTN, Portmann Bet al. Relapse following treatment withdrawal in patients with autoimmune chronic active hepatitis.Hepatology 1983; 3: 685–689.Google Scholar
  33. 33.
    Dubois RS, Silverman A. Treatment of chronic active hepatitis in children.Postgrad Med J 1974; 50: 386–391.PubMedCrossRefGoogle Scholar
  34. 34.
    Arasu TS, Wyllie R, Hatch TF, Fitzgerald JF. Management of chronic agressive hepatitis in children and adolescents.J Pediatr 1979; 95: 514–522.PubMedCrossRefGoogle Scholar
  35. 35.
    Maggiore G, Bernard O, Hadchouel Met al. Treatment of autoimmune chronic active hepatitis in childhood.J. Pediatr 1984; 104: 839–844.PubMedCrossRefGoogle Scholar
  36. 36.
    Naggiore G, Veber F, Bernard Oet al. Autoimmune hepatitis associated with antiactine antibodies in children and adolescents.J Pediatr Gastroenterol Nutrit 1993; 17: 376–381.Google Scholar
  37. 37.
    Stellon AJ, Keating JJ, Johnson PJet al. Maintenance of remisdsion in autoimmune chronic active hepatitis with azathioprine after corticosteroid withdrawal.Hepatology 1988; 8: 781–784.PubMedCrossRefGoogle Scholar
  38. 38.
    Hyams JS, Ballow M, Leichtner AM. Cyclosporine treatment of autoimmune chronic active hepatitis.Gastroenterology 1987; 93: 890–893.PubMedGoogle Scholar
  39. 39.
    Treem WR, Hyams JS. Cyclosporine therapy for gastrointestinal disease.J Pediatr Gastroenterol Nutrit 1994; 18: 270–278.CrossRefGoogle Scholar

Copyright information

© Dr. K C Chaudhuri Foundation 1995

Authors and Affiliations

  • Eve A. Roberts
    • 1
  1. 1.Division of Gastroenterology and Nutrition, The Hospital for Sick ChildrenUniversity of TorontoTorontoCanada

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