Liver

• Chronic progressive inflammation of the liver, untreated, eventually leads to cirrhosis of the liver
• Hypergammaglobulinaemia
• Elevated transaminases
• Circulating auto-antibodies

Chronic autoimmune disorders of the liver include:

• Autoimmune hepatitis (AIH)
• Primary biliary cirrhosis (PBC)
• Primary sclerosing cholangitis (PSC)
• Autoimmune cholangiopathy
• Overlap syndromes

Autoimmune hepatitis can be divided into 2 major types, depending on seropositivity for antinuclear antibodies (ANA) and/or smooth muscle (ASM) or antibodies to liver kidney microsomes (LKM). A further division of AIH, namely, type 3 has been suggested by some experts.

AIH type 1:

• ASM and/or ANA
• Target antigen: Actin
• Hepatitis B infection could be one cause
• Incidence: Incidence: 90%, affects younger females

AIH type 2:

• Liver kidney microsomes (LKM) and/or anti-lver cytosol-1 (LC-1)
• Target antigen: Cytochrome P450 (LKM) and 56 kDa Formininotransferase- cylodeaminase (LC-1)
• Hepatitis C could be responsible in about 7% of AIH
• Incidence: 90%, affects younger females

AIH type 3:

• Anti-SLA/LP (in absence of any other liver disease markers)

Primary biliary cirrhosis

• Anti-mitochondrial antibodies (AMA)
• Target antigen: M2 (pyruvate dehydrogenase complex)
• Disease: primary biliary cirrhosis
• Infection is thought to trigger the disease
• Incidence: 90%, affects older females

Primary sclerosing cholangitis (PSC)

• pANCA (65%), ASM (11%) and/or ANA (30%)

Autoimmune cholangiopathy

• AMA negative but pANCA, ANA and ASM may be present

Overlap syndromes

• Two overlapping diseases, for example, combination of PBC and AIH
• ASM and/or ANA and AMA