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[Autoimmune chronic active hepatitis]

E L Renner1

  • 1Institut für klinische Pharmakologie, Universität Bern.

Therapeutische Umschau. Revue Therapeutique
|February 1, 1993
PubMed
Summary
This summary is machine-generated.

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Autoimmune chronic active hepatitis (ACAH) is a rare liver disease affecting women, characterized by autoantibodies and often diagnosed late. Immunosuppressive therapy improves survival but may require long-term treatment.

Area of Science:

  • Hepatology
  • Immunology
  • Internal Medicine

Context:

  • Autoimmune chronic active hepatitis (ACAH) is a rare liver condition.
  • It primarily affects women and presents with a bimodal age distribution.
  • Diagnosis is based on negative viral markers, hypergammaglobulinemia, and autoantibodies.

Purpose:

  • To describe the characteristics, pathogenesis, prognosis, and treatment of ACAH.
  • To highlight the heterogeneity and unknown causes of the disease.
  • To discuss the role of immunosuppressive therapy and liver transplantation.

Summary:

  • ACAH presents with non-specific symptoms or as an asymptomatic stage, often with accompanying autoimmune diseases.
  • Histology reveals chronic inflammation and piece-meal necrosis.

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  • Potential triggers include exogenous substances and hepatitis C virus on a permissive genetic background.

    • Impact:

    • Untreated ACAH is progressive, leading to cirrhosis and high mortality.
    • Immunosuppressive therapy, primarily corticosteroids, can induce remission but often requires indefinite treatment.
    • Liver transplantation is the only option for end-stage disease, with therapy improving 5-year survival rates to over 80%.