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Key Features

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Essentials of Diagnosis
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  • Acute or chronic hepatitis

  • Hypergammaglobulinemia

  • Positive antinuclear antibodies (ANA), anti–smooth muscle antibodies (ASMA), anti–liver-kidney microsomal (LKM) antibodies, anti-actin antibodies, or anti–soluble liver antigen antibodies (SLA)

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General Considerations
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  • Progressive inflammatory disorder of unknown etiology

  • Characterized histologically by portal tract inflammation that extends into the parenchyma; serologically by the presence of nonorgan-specific autoantibodies; biochemically by elevated aminotransferases and serum IgG; and clinically by response to immunosuppressive treatment in the absence of other known causes of liver disease

  • A family history of autoimmune diseases is present in approximately 40% of cases

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Clinical Findings

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Symptoms and Signs
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  • Often asymptomatic early in the disease process

  • Lethargy as well as malaise

  • Jaundice

  • Recurrent fevers

  • Abdominal pain or distention

  • Other complaints at the time of presentation may include

    • Recurrent rash

    • Arthritis

    • Chronic diarrhea

    • Amenorrhea

  • Hepatomegaly or splenomegaly may be found on examination

  • In more advanced cases, jaundice and ascites may develop

  • Cutaneous signs of chronic liver disease may be noted

    • Spider angiomas

    • Palmar erythema

    • Digital clubbing

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Differential Diagnosis
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  • Hepatitis B

  • Hepatitis C

  • Steatohepatitis

  • Wilson disease

  • α1-Antitrypsin deficiency

  • Primary sclerosing cholangitis

  • Drug-induced chronic hepatitis

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Diagnosis

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Laboratory Findings
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  • Moderate elevations of serum AST, ALT

  • Variable elevations of alkaline phosphatase, bilirubin, and total IgG

  • Liver biopsy

    • Remains the gold standard in diagnosis

    • Reveals the typical histologic picture of interface hepatitis

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Treatment

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  • Corticosteroids (prednisone, 2 mg/kg/d maximum 60 mg) as induction therapy decreases the mortality rate during the early active phase of the disease

  • Azathioprine or 6-mercaptopurine (6-MP), 1–2 mg/kg/d, as maintenance therapy

    • Valuable in decreasing the side effects of long-term corticosteroid therapy

    • Should not be used alone during the induction therapy

  • Thiopurine methyltransferase activity in red blood cells or genotype should be assessed prior to starting azathioprine or 6-MP to prevent extremely high blood levels and severe bone marrow toxicity

  • In type 1 autoimmune hepatitis

    • Corticosteroids are reduced over a 3- to 6-month period

    • Azathioprine is continued for at least 1–2 years

    • If AST and ALT have been consistently normal, tapering therapy can be considered

    • Liver biopsy must be performed before stopping azathioprine or 6-MP therapy; if any inflammation persists, then azathioprine or 6-MP is continued

    • Most pediatric patients require long-term azathioprine or 6-MP therapy

    • Relapses are treated with a course of corticosteroids

  • In type 2 autoimmune hepatitis, patients must continue taking azathioprine and low-dose corticosteroids

  • Cyclosporine, tacrolimus, or methotrexate may be helpful in poorly responsive cases

  • Mycophenolate mofetil can be substituted for azathioprine or 6-MP

  • Liver transplantation indications

    • Disease progresses to decompensated cirrhosis

    • Acute liver failure that does not respond to corticosteroid therapy

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Outcome

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Complications
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  • Untreated disease eventually results in cirrhosis, with complications of portal hypertension and ...

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