Primary Biliary Cirrhosis

Question 1

Definition of PBC

Answer 1

• Immune system attacks small bile ducts within the liver
• First part to be affected are the intralobular ducts
• Causes obstruction - cholestasis
• Back pressure leads to fibrosis, cirrhosis and liver failure
• Associated with other autoimmune diseases and rheumatological conditions - more common in middle aged women

Question 2

Presentation of PBC

Answer 2

• Fatigue
• Jaundice
• Pruritis
• GI disturbance
• Pale stools
• Xanthoma and xnthelasma
• Signs of cirrhosis and failure

Question 3

Diagnosis of PBC

Answer 3

• ALP is first liver enzyme to be raised (as with most obstructive pathology)
• Other liver enzymes and bilirubin raised later in disease
• Anti-mitochondrial antibodies are most specific to PBC
• Raised ESR and IgM
• Liver biopsy for staging the disease

Question 4

Management of PBC

Answer 4

• Ursodeoxcholic acid reduces intestinal absorption of cholesterol
• Colesturamine is a bile acid sequestrate
• Liver transplant
• Immunosuppression (i.e. steroids)