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For further information, see CMDT Part 16-07: Autoimmune Hepatitis

Key Features

Essentials of Diagnosis

  • Chronic hepatitis with high serum globulins and characteristic liver histology

  • Antinuclear antibody (ANA) and/or smooth muscle antibody positive

  • Usually occurs in young to middle-aged women

  • Responds to corticosteroids

General Considerations

  • The onset is usually insidious

  • About 20% of cases present with acute hepatitis (and occasionally acute liver failure)

  • Some cases follow

    • A viral illness, such as

      • Hepatitis A

      • Epstein-Barr infection

      • Measles

    • Exposure to a drug or toxin, such as

      • Nitrofurantoin

      • Minocycline

      • Hydralazine

      • Methyldopa

      • Infliximab

  • Patients with autoimmune hepatitis are at increased risk for cirrhosis which, in turn, increases the risk of hepatocellular carcinoma (at a rate of about 1% per year)

  • The risk of autoimmune hepatitis is increased in first-degree relatives of affected patients

  • Simplified diagnostic criteria are based on

    • Detection of autoantibodies (1 or 2 points depending on titers)

    • Elevated IgG levels (1 or 2 points depending on levels)

    • Characteristic histologic features (1 or 2 points depending on how typical the features are)

    • Exclusion of viral hepatitis (2 points)

  • Score of 6 indicates probable and a score of 7 indicates definite autoimmune hepatitis

  • Diagnostic criteria for an overlap of autoimmune hepatitis and primary biliary cholangitis ("Paris criteria") have been proposed

Demographics

  • Though usually a disease of young women, autoimmune hepatitis can occur in either sex at any age

  • Affected younger persons are often positive for HLA-B8 and -DR3; older patients are often positive for HLA-DR4

  • The principal susceptibility allele among white Americans and northern Europeans is HLA DRB1*0301; HLA DRB1*0401 is a secondary but independent risk factor

Clinical Findings

Symptoms and Signs

  • Typically, a healthy-appearing young woman with multiple spider telangiectasias, cutaneous striae, acne, hirsutism, and hepatomegaly

  • Amenorrhea may be a presenting feature

  • Frequency of depression appears to be increased

  • Extrahepatic features

    • Arthritis

    • Sjögren syndrome

    • Thyroiditis

    • Nephritis

    • Ulcerative colitis

    • Coombs-positive hemolytic anemia

Differential Diagnosis

  • Chronic viral hepatitis

  • Primary biliary cholangitis

  • Primary sclerosing cholangitis

  • Wilson disease

  • Hemochromatosis

  • Drug-induced liver disease

  • α1-Antiprotease (antitrypsin) deficiency

Diagnosis

Laboratory Tests

  • The serum bilirubin is usually increased, but 20% are anicteric

  • Serum aminotransferase levels may be > 1000 units/L

  • Type I (classic) autoimmune hepatitis

    • ANA or smooth muscle antibodies (either or both) are usually detected in serum

    • Serum γ-globulin levels are typically elevated (up to 5–6 g/dL [0.05-0.06 g/L])

      • In this setting, the enzyme immunoassay for antibody to hepatitis C virus may be falsely positive

      • Other antibodies, including antineutrophil cytoplasmic antibodies (ANCA), may be found

  • Type II

    • Seen more often in Europe in girls under age 14

    • Characterized by circulating antibodies to liver–kidney microsome type 1 (anti-LKM1)— directed against cytochrome P450 2D6 — or anti-liver cytosol type 1 —directed against formiminotransferase — ...

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