Autoimmune Liver Disease

  • Mikio ZeniyaEmail author
  • Masaki Iwai
  • Arief A. Suriawinata


Autoimmunity of the human body can be directed toward the hepatocytes or toward the bile ducts. Autoimmune disease affecting the hepatocytes (with elevation of hepatocellular enzymes—AST, ALT) is called autoimmune hepatitis (AIH), whereas the classical example of autoimmune disease affecting small bile ducts is called primary biliary cholangitis (PBC). The latter is associated with elevation of cholestatic liver enzyme (ALP, gamma-GT). The role of autoimmunity in primary sclerosing cholangitis (PSC) affecting the large bile ducts is rather unclear, although in recent years a variant of PSC is certainly caused by IgG4-mediated bile duct injury. Finally, sometimes features of both AIH and PBC or PSC can be present, producing so-called overlap syndrome.


Autoimmune hepatitis Primary biliary cholangitis (primary biliary cirrhosis) Primary sclerosing cholangitis Autoantibody Chronic nonsuppurative destructive cholangitis Human leucocyte antigen Overlap syndrome Graft-versus-host reaction 



Autoimmune hepatitis


Alkaline phosphatase


Anti-mitochondrial antibody


Antinuclear antibody


Anti-smooth muscle antibody


Chronic nonsuppurative destructive cholangitis


Endoscopic retrograde cholangiopancreatography




Glutamic oxaloacetic transaminase


Glutamic pyruvic transaminase


Graft-versus-host disease


Human leucocyte antigen




Magnetic resonance cholangiopancreatography


Primary biliary cholangitis


Primary sclerosing cholangitis


Ursodeoxycholic acid



Prof. Dirk J. van Leeuwen was a coauthor of the first edition of this chapter.


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Copyright information

© Springer Nature Singapore Pte Ltd. 2019

Authors and Affiliations

  • Mikio Zeniya
    • 1
    Email author
  • Masaki Iwai
    • 2
  • Arief A. Suriawinata
    • 3
  1. 1.TokyoJapan
  2. 2.KyotoJapan
  3. 3.New HampshireUSA

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