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  • Autoimmune hepatitis (AIH) is a disease of unknown etiology and is characterized by increased serum aminotransferases, presence of lymphoplasmacytic infiltration and interface hepatitis on histology, autoantibodies, and hypergammaglobulinemia.1

  • Clinical presentation can vary from mild asymptomatic disease to acute fulminant liver failure.2


  • AIH affects all ages and both genders but predominantly is found in women with a preponderance to males of 3.5:1 in the United States.3

  • It is relatively rare and affects 100,000 to 200,000 persons in the United States and accounts for 2% to 3% of the pediatric and 4% to 6% of the adult liver transplants performed in the United States and Europe.1

  • Prevalence of disease varies among races, though it has been associated with a genetic predisposition. Prevalence is the highest among North American and Northern European white persons with HLA-DRB1*03 and HLA-DRB*04.4


  • AIH develops due to genetic predisposition, antigenic exposures, and immunologic activation of a T-cell–mediated cascade, which leads to progressive inflammatory and fibrotic liver disease.5

  • Potential triggers include viruses, drugs, and herbal agents. The time between exposure and onset of disease can vary.6

  • Genetic susceptibility for AIH results from human leukocyte antigen (HLA) genes that reside on the major histocompatibility complex (MHC) located on chromosome 6.3,7

  • Type 1 AIH is associated with the HLA-DR3 serotype and is particularly seen in white patients. This disease is more common in the early-onset, severe form of AIH and occurs in girls and young women. In contrast, HLA-DR4 is more common in adults and may have a higher incidence of extrahepatic, milder disease with better response to corticosteroids.5

  • Type 2 AIH is rare and is associated with the HLA-DRB1 and HLA-DQB1 alleles.8

  • Polymorphisms in the tumor necrosis factor (TNF) gene, cytotoxic T lymphocyte antigen 4 gene (CTLA4), and Fas gene promoter have been associated with increased reactivity of the immune system, disease severity, and early progression to cirrhosis.9–11

  • Liver cell destruction occurs via cell-mediated cytotoxicity, antibody-dependent cell-mediated cytotoxicity, or a combination of both depending on the immune cascade.12,13


  • Major symptoms of AIH include fatigue and arthralgia. Jaundice indicates an acute severe disease or a chronic process with advanced fibrosis.5

  • Acute or acute severe (fulminant) presentation may resemble acute viral or toxic hepatitis. Centrilobular hemorrhagic necrosis with lymphoblastic infiltration, lymphoid aggregates, or plasma cell infiltration is the main histologic finding (Fig. 8-1).14


Histopathology of centrilobular zone 3 necrosis with hepatocyte rosettes. (H&E, ×200)6


  • The original scoring system (Table 8-1) that was proposed by the International Autoimmune Hepatitis Group (IAIHG) provides a score to reflect the strength of the diagnosis prior to ...

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