Primary Biliary Cirrhosis (PBC)

• Interlobular bile ducts are damaged by chronic granulomatous inflammation causing progressive cholestasis, cirrhosis and portal hypertension
• Autoimmune F>>M
• Present in the 50′s

Clinical features

• Often found by routine LFTs showing increased Alk Phos
• Lethargy and puritis can occur followed by jaundice
• Signs – jaundice, skin pigmentation, xanthelasma, xanthomata, hepatomegaly, splenomegaly
• Complications;
  • Osteoporosis
  • Malabsorption of fat sol vitamins – osteomalacia, coagulopathy
  • Portal hypertension, ascites, variceal haemorrhage, hepatic encephalopathy, HCC

Tests

• Raised Alk Phos, ?-GT, mildly elevated AST, ALT, late disease raised bilirubin and reduced albumin, increased PT
• 98% are antimitochondial M2 subtype positive
• Ig is raised especially IgM
• TSH and cholesterol may be raised
• Radiology – ultrasound and ERCP to extrahepatic cholestasis
• Liver biopsy – granulomas around the bile duct

Treatment

• Puritis – cholestyramine
• Diarrhoea – codeine phosphate
• Give fat sol vitamins
• Ursodeoxycholic acid (a bile acid)
• Liver transplantation

Prognosis

• Once jaundice develops survival is <2yr