Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ Key Features +++ Essentials of Diagnosis ++ 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) +++ General Considerations ++ 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 +++ Clinical Findings +++ Symptoms and Signs ++ 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 +++ Differential Diagnosis ++ Hepatitis B Hepatitis C Steatohepatitis Wilson disease α1-Antitrypsin deficiency Primary sclerosing cholangitis Drug-induced chronic hepatitis +++ Diagnosis +++ Laboratory Findings ++ 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 +++ Treatment ++ 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 +++ Outcome +++ Complications ++ Untreated ... Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth