Autoimmune hepatitis

Autoimmune hepatitis is condition of unknown aetiology which is most commonly seen in young females. Recognised associations include other autoimmune disorders, hypergammaglobulinaemia and HLA B8, DR3. Three types of autoimmune hepatitis have been characterised according to the types of circulating antibodies present

Affects both adults and children | Anti-liver/kidney microsomal type 1 antibodies (LKM1)

Affects children only | Soluble liver-kidney antigen

Affects adults in middle-age |

Features

• may present with signs of chronic liver disease
• acute hepatitis: fever, jaundice etc (only 25% present in this way)
• amenorrhoea (common)
• ANA/SMA/LKM1 antibodies, raised IgG levels
• liver biopsy: inflammation extending beyond limiting plate 'piecemeal necrosis', bridging necrosis

Management

• steroids, other immunosuppressants e.g. azathioprine
• liver transplantation

Type I - Young woman + signs of liver inflammation + ANA positive + ASMA +

Type II - Children Europe > US, anti LKM-1 +, usually ASMA and ANA -

More specific tests = liver-kidney microsomal antibodies, high gamma globulin (IgG) and anti-smooth muscle antibodies

The most accurate test = liver biopsy

Tx

prednisone and/or azathioprine