Primary biliary cirrhosis PBC

Primary biliary cirrhosis
  • Facts:
    Autoimmune disorder
    Destruction of intrahepatic ducts and cholestasis
    More common in females ( 9:1 )
    MCC of ductopenia in adults
  • History / PE:
    Malabsorption of fat soluble vitamins
    Portal HTN
  • Diagnosis:
    Elevated alkaline phosphatase
    Anti-mitochodrial antibodies
    Xanthelasma (hypercholesterolemia)
  • Treatment:
    Ursodeoxycholic acid (slows progression of disease)
    Methotrexate and colchicine
    Cholestyramine (pruritis)
    Liver transplantation
  • Associated With:
    Raynaud's phenomenon
    CREST syndrome
  • Differential Diagnosis:
    Vanishing bile duct syndrome
    Primary sclerosing cholangitis

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July 26th 2010