Autoimmune Hepatitis

  • F. Wilson Jackson
  • Raymond A. Rubin
Part of the Current Clinical Practice book series (CCP)


Autoimmune hepatitis (AIH) is a progressive inflammatory liver disease which affects females four times as commonly as males. The disease predominantly affects women in their third through fifth decades. Presenting symptoms usually include fatigue, malaise, oligomenorrhea, and sometimes jaundice. AIH is characterized by persistent elevations in serum tran-saminases, increased serum IgG, and histologic evidence of piecemeal necrosis, often with predominant plasma cell infiltration of the portal tracts. Classically, patients with AIH display high titers of antinuclear antibody (ANA) and anti-smooth muscle antibody (ASMA).


Autoimmune Hepatitis Chronic Active Hepatitis Fulminant Hepatic Failure Portal Tract Chronic Active Liver Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Waldenstrom J, Leber B. Bluproteine und Nahrungseiweiss. Deutsche Gesellschaft Zeitschrift für Verdaungs — und Staffwechselkrankheiten, 1950;15:113–119.Google Scholar
  2. 2.
    Mackay JR, Taft LI, Cowling DC. Lupoid hepatitis. Lancet 1956;2:1323–1326.CrossRefGoogle Scholar
  3. 3.
    Soloway RD, Summerskill WHJ, Baggenstoss AH, et. al. “Lupoid” hepatitis, a nonentity in the spectrum of chronic active liver disease. Gastroenterology 1972;63:458.PubMedGoogle Scholar
  4. 4.
    Lenzi M, Ballordini G, Fusconi M. Type 2 autoimmune hepatitis and hepatitis C virus infection. Lancet 1990;355:258–259.CrossRefGoogle Scholar
  5. 5.
    Johnson PJ, McFarlance IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology 1993;18:998–1005.PubMedCrossRefGoogle Scholar
  6. 6.
    Donaldson PT, Doherty DG, Hayllar KM, McFarlane IG, Johnson PJ, Williams R. Susceptibility to autoimmune chronic active hepatitis: human leukocyte antigens DR-4 and A1-B8-DR-3 are independent risk factors. Hepatology 1991;13:701–706.PubMedCrossRefGoogle Scholar
  7. 7.
    Czaja AJ, Davis GL, Ludwig J. Autoimmune features as determinants of prognosis in steroid-treated chronic active hepatitis of uncertain etiology. Gastroenterology 1983;85:713.PubMedGoogle Scholar
  8. 8.
    Donaldson P, Daherty D, Underhill J, Williams R. The molecular genetics of autoimmune liver disease. Hepatology 1994;20:225–239.PubMedCrossRefGoogle Scholar
  9. 9.
    Lidman K, Biberfield G, Fagraeus A. Anti-actin specificity of human smooth muscle antibodies in chronic active hepatitis. Clin Exp Immunol 1976;24:266–272.PubMedGoogle Scholar
  10. 10.
    Targan SR, Landers C, Vidrich A, Czaja AJ. High titer antineutrophil cytoplasmic antibodies in Type 1 autoimmune hepatitis. Gastroenterology 1995;108:1159-11-66.Google Scholar
  11. 11.
    Czaja AJ. Natural history, clinical features, and treatment of autoimmune hepatitis. Sem Liver Dis 1984;4:1–12.CrossRefGoogle Scholar
  12. 12.
    Czaja AJ, Manns MP, Homburger HA. Frequency and significance of antibodies to liver/kidney microsomes type 1 in adults with chronic active hepatitis. Gastroenterology 1992; 103:160–165.Google Scholar
  13. 13.
    Hornberg JC, Abuaf N, Bernard O. Chronic active hepatitis associated with anti liver/kidney microsome antibody type 1: a second type of “autoimmune” hepatitis. Hepatology 1987;7:1333–1339.CrossRefGoogle Scholar
  14. 14.
    Manns MP, Griffin KJ, Sullivan KF, Johnson EF. LKM-1 autoantibodies recognize a short linear sequence in P450IID6, a cytochrome P-450 monooxygenase. J Clin Invest 1991;88:1370–1378.PubMedCrossRefGoogle Scholar
  15. 15.
    Manns M, Gerkin G, Kyriatsoulis. Characteristics of a new subgroup of autoimmune chronic active hepatitis by autoantibodies against a soluble liver antigen. Lancet 1987; 1:62.Google Scholar
  16. 16.
    Porta G, DaCosta Gayotto LC, Alvarez F. Anti-liver-kidney microsome antibody-positive autoimmune hepatitis presenting as fulminant liver failure. J Ped Gastroenterol Nutr 1990; 11:138.CrossRefGoogle Scholar
  17. 17.
    Davis GL, Czaja AJ, Baggenstoss AH. Prognostic and therapeutic implications of extreme serum aminotrans-ferase elevation in chronic active hepatitis. Mayo Clin Proc 1982;57:303.PubMedGoogle Scholar
  18. 18.
    Kenny RP, Czaja AJ, Ludwig. Frequency and significance of antimitichondrial antibodies in severe chronic active hepatitis. Dig Dis Sci 1986;31:705.PubMedCrossRefGoogle Scholar
  19. 19.
    Bach N, Thung SN, Schaffner F. The histological features of chronic hepatitis C and autoimmune hepatitis: a comparative analysis. Hepatology 1992; 15:572–577.PubMedCrossRefGoogle Scholar
  20. 20.
    Summerskill WHJ. Chronic active liver disease reexamined: prognosis hopeful. Gastroenterology 1974;66:450–464.PubMedGoogle Scholar
  21. 21.
    Czaja AJ. Treatment strategies in chronic active hepatitis. In: Czaja AJ, Dickson ER, eds. Chronic active hepatitis: the Mayo Clinic experience. Marcel Dekker, New York, 1986, pp. 247–267, 269-283.Google Scholar
  22. 22.
    Cook AG, Mulligan R, Sherlock S. Controlled prospective trial of corticosteroid therapy in chronic active hepatitis. Q J Med 1971;158:159–185.Google Scholar
  23. 23.
    Murray-Lyon IM, Stren RB, Williams, R. Controlled trial of prednisone and azathioprine in active chronic hepatitis. Lancet 1973;1:735–737.PubMedCrossRefGoogle Scholar
  24. 24.
    Soloway RD, Summerskill WHJ, Baggenstoss AH. 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
  25. 25.
    Summerskill WHJ, Korman MG, Ammon HV. Prednisone for chronic active liver disease: dose titration, standard dose, and combination with azathioprine compared. Gut 1975; 16:876.PubMedCrossRefGoogle Scholar
  26. 26.
    Johnson PJ, McFarlane IG, Williams R. Azathioprine for the long-term maintenance of remission in autoimmune hepatitis. N Engl J Med, 1995;333:958–963.PubMedCrossRefGoogle Scholar
  27. 27.
    Person JL, McHutchenson JG, Fong TL, Redeker AG. A case of cyclosporin-sensitive, steroid-resistant, autoimmune chronic active hepatitis. J Clin Gastroenterol 1993; 17:317–320.PubMedCrossRefGoogle Scholar
  28. 28.
    Hyams JS, Ballow M, Leichtner AM. Cyclosporin treatment of autoimmune chronic active hepatitis. Gastroenterology 1987;93:890–893.PubMedGoogle Scholar
  29. 29.
    Alstead EM, Ritchie JK, Lennard-Jones. Safety of azathioprine in pregnancy in inflammatory bowel disease. Gastroenterology 1990;99:443–446.PubMedGoogle Scholar
  30. 30.
    Sanchez-Urdazpal L, Czaja AJ, van Hoek B. Prognostic features and role of liver transplantation in severe corticosteroid-treated autoimmune chronic active hepatitis. Hepatology 1992; 15:215.PubMedCrossRefGoogle Scholar
  31. 31.
    Neuberger J, Portman B, Calne R. Recurrence of autoimmune chronic active hepatitis following orthotopic liver grafting. Transplantation 1984;37:363.PubMedCrossRefGoogle Scholar
  32. 32.
    Burroughs AK, Bassendine MF, Thomas HC, Sherlock S. Primary liver cancer in autoimmune chronic liver disease. Brit Med J 1981;282:273.CrossRefGoogle Scholar
  33. 33.
    Kemeny MJ, O’Hanlon G, Gregory PB. Asymptomatic chronic active hepatitis—prognosis and treatment. (Abstract) Gastroenterology 1984;86:1325.Google Scholar
  34. 34.
    Czaja AJ, Davis GL, Ludwig J. Complete resolution of inflammatory activity following corticosteroid treatment of HBsAg-negative chronic active hepatitis. Hepatology 1984;4:622.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1998

Authors and Affiliations

  • F. Wilson Jackson
  • Raymond A. Rubin

There are no affiliations available

Personalised recommendations