Primary biliary cirrhosis PBC

• Facts:
  Autoimmune disorder
  Destruction of intrahepatic ducts and cholestasis
  More common in females ( 9:1 )
  MCC of ductopenia in adults
• History / PE:
  Jaundice
  Pruritis
  Malabsorption of fat soluble vitamins
  Portal HTN
  Osteopenia
  
• 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:
  Hypothyroidism
  Raynaud's phenomenon
  CREST syndrome
• Differential Diagnosis:
  Vanishing bile duct syndrome
  Primary sclerosing cholangitis