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Autoimmune hepatitis: Diagnosis, treatment, and prognosis

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Abstract

Autoimmune hepatitis (AIH) is an idiopathic hepatitis of unknown cause characterized by histologic evidence of chronic inflammation, liver autoantibodies, and increased gamma globulins in the serum. The clinical manifestations of AIH have been known since the 1950s and are essentially unchanged over the past 50 years. The cornerstone of therapy for AIH is immunosuppression using prednisone and azathioprine, with the vast majority of patients achieving clinical remission. This review provides a concise summary of the diagnostic criteria for AIH, describes its proposed pathogenic mechanisms and epidemiology, and offers an algorithmic approach to its initial treatment and follow-up.

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References and Recommended Reading

  1. Czaja AJ, Donaldson PT: Genetic susceptibilities for immune expression and liver cell injury in autoimmune hepatitis. Immunol Rev 2000, 174:250–259.

    Article  PubMed  CAS  Google Scholar 

  2. Czaja AJ: Understanding the pathogenesis of autoimmune hepatitis. Am J Gastroenterol 2001, 96:1224–1231.

    Article  PubMed  CAS  Google Scholar 

  3. Boberg KM, Aadland E, Jahnsen J, et al.: Incidence and prevalence of primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune hepatitis in a Norwegian population. Scand J Gastroenterol 1998, 33:99–103.

    Article  PubMed  CAS  Google Scholar 

  4. Wiesner RH, Demetris AJ, Belle SH, et al.: Acute hepatic allograft rejection: incidence, risk factors, and impact on outcome. Hepatology 1998, 28:638–645.

    Article  PubMed  CAS  Google Scholar 

  5. Czaja AJ: Frequency and significance of phenotypes for alpha1-antitrypsin deficiency in type 1 autoimmune hepatitis. Digest Diseases Sci 1998, 43:1725–1731.

    Article  CAS  Google Scholar 

  6. Soloway RD, Summerskill WH, Baggenstoss AH, Schoenfield LJ: "Lupoid" hepatitis, a nonentity in the spectrum of chronic active liver disease. Gastroenterology 1972, 63:458–65.

    PubMed  CAS  Google Scholar 

  7. Murray-Lyon IM, Stern RB, Williams R: Controlled trial of prednisone and azathioprine in active chronic hepatitis. Lancet 1973, 1(7806):735–737.

    Article  PubMed  CAS  Google Scholar 

  8. Gonzalez-Koch A, Czaja AJ, Carpenter HA, et al.: Recurrent autoimmune hepatitis after orthotopic liver transplantation. Liver Transpl 2001, 7:302–310.

    Article  PubMed  CAS  Google Scholar 

  9. Johnson PJ, McFarlane IG: Meeting report: International Autoimmune Hepatitis Group. Hepatology 1993, 18:998–1005. An important article detailing the findings of an international consensus group on the definition of treatment of AIH.

    Article  PubMed  CAS  Google Scholar 

  10. 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. An important article from the International Autoimmune Hepatitis Group that includes the specifics of the currently accepted scoring system for the diagnosis of AIH.

    Article  PubMed  CAS  Google Scholar 

  11. Czaja AJ: Frequency and nature of the variant syndromes of autoimmune liver disease. Hepatology 1998, 28:360–365. A classic paper written by one of the experts in the field of AIH describing the variant syndromes of autoimmune liver disease. This paper contains descriptions of the various overlap syndromes that can complicate the diagnosis of AIH.

    Article  PubMed  CAS  Google Scholar 

  12. Czaja AJ, Carpenter HA, Santrach PJ, Moore SB: Autoimmune cholangitis within the spectrum of autoimmune liver disease [see comment]. Hepatology 2000, 31:1231–1238.

    Article  PubMed  CAS  Google Scholar 

  13. Czaja AJ, Carpenter HA: Autoimmune hepatitis with incidental histologic features of bile duct injury. Hepatology 2001, 34(4 Pt 1):659–665.

    Article  PubMed  CAS  Google Scholar 

  14. Ben-Ari Z, Czaja AJ: Autoimmune hepatitis and its variant syndromes. Gut 2001, 49:589–594.

    Article  PubMed  CAS  Google Scholar 

  15. Czaja AJ, Carpenter HA: Sensitivity, specificity, and predictability of biopsy interpretations in chronic hepatitis [see comment]. Gastroenterology 1993, 105:1824–1832.

    PubMed  CAS  Google Scholar 

  16. Meyer O, Meyer O, Abuaf N, et al.: Anti-mitochondrial type 5 antibodies and anti-cardiolipin antibodies in systemic lupus erythematosus and auto-immune diseases. Clin Exp Immunol 1987, 69:485–492.

    PubMed  CAS  Google Scholar 

  17. Czaja AJ, Manns MP: The validity and importance of subtypes in autoimmune hepatitis: a point of view. Am J Gastroenterol 1995, 90:1206–1211. A nice review article looking at the definition and importance of subtype analysis in patients with AIH. Although not all experts believe that subtypes are clinically important, this review article describes the pros and cons of determining subtypes.

    PubMed  CAS  Google Scholar 

  18. Czaja AJ: Behavior and significance of autoantibodies in type 1 autoimmune hepatitis. J Hepatol 1999, 30:394–401.

    Article  PubMed  CAS  Google Scholar 

  19. Czaja AJ, Freese DK, American Association for the Study of Liver Diseases: Diagnosis and treatment of autoimmune hepatitis. Hepatology 2002, 36:479–497. A very important paper with the state-of-the-art description of the disease published by the American Association for the Study of Liver Disease.

    Article  PubMed  Google Scholar 

  20. Czaja AJ, Kruger M, Santrach PJ, et al.: Genetic distinctions between types 1 and 2 autoimmune hepatitis [see comment]. Am J Gastroenterol 1997, 92:2197–2200.

    PubMed  CAS  Google Scholar 

  21. Bittencourt PL, Goldberg AC, Cancado EL, et al.: Genetic heterogeneity in susceptibility to autoimmune hepatitis types 1 and 2. Am J Gastroenterol 1999, 94:1906–1913.

    Article  PubMed  CAS  Google Scholar 

  22. Cook GC, Mulligan R, Sherlock S: Controlled prospective trial of corticosteroid therapy in active chronic hepatitis. QJM 1971, 40:159–185. A classic paper reporting one of the first trials of corticosteroid therapy in patients believed to have AIH.

    PubMed  CAS  Google Scholar 

  23. Uribe M, Go VL, Kluge D: Prednisone for chronic active hepatitis: pharmacokinetics and serum binding in patients with chronic active hepatitis and steroid major side effects. J Clin Gastroenterol 1984, 6:331–335.

    PubMed  CAS  Google Scholar 

  24. Czaja AJ: Drug therapy in the management of type 1 autoimmune hepatitis. Drugs 1999, 57:49–68. An excellent review by one of the experts in AIH describing his approach to its pharmacologic management.

    Article  PubMed  CAS  Google Scholar 

  25. McCullough AJ, Czaja AJ: Relapse following treatment withdrawal in autoimmune chronic active hepatitis. Hepatology 1984, 4:747–748.

    PubMed  CAS  Google Scholar 

  26. Czaja AJ, Wolf AM, Baggenstoss AH: Laboratory assessment of severe chronic active liver disease during and after corticosteroid therapy: correlation of serum transaminase and gamma globulin levels with histologic features. Gastroenterology 1981, 80:687–692.

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Gastroenterology and Hepatology, Saint Louis University Health Sciences Center, 3635 Vista Avenue at Grand Boulevard, 63110-0250, St. Louis, MO, USA

    Bruce A. Luxon MD, PhD

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  1. Bruce A. Luxon MD, PhD
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Correspondence to Bruce A. Luxon MD, PhD.

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Luxon, B.A. Autoimmune hepatitis: Diagnosis, treatment, and prognosis. Curr Gastroenterol Rep 8, 83–88 (2006). https://doi.org/10.1007/s11894-006-0068-7

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  • DOI: https://doi.org/10.1007/s11894-006-0068-7

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