Primary Biliary Cirrhosis/Cholangitis (PBC)

Question 1

Describe the presumed aetiology of PBC

Answer 1

• autoimmune condition. - Interlobular bile ducts become damaged causing progressive cholestasis

Question 2

What patient demographic usually develops PBC

Answer 2

middle aged females

Female:Male ratio = 9:1

Question 3

Other conditions associated with PBC

Answer 3

Sjogren’s syndrome
rheumatoid arthritis
systemic sclerosis
thyroid disease

Question 4

Clinical features of PBC

Answer 4

May be asymptomatic (Raised ALP on routine LFTs)
- fatigue
- pruritus
- jaundice
- RUQ pain
- xanthelasma/xanthomata
- clubbing
- hepatosplenomegaly

Question 5

What antibodies are commonly seen in PBC

Answer 5

anti-mitochondrial antibodies

raised serum IgM

Question 6

Imaging used to rule out extrahepatic biliary obstruction prior to diagnosis

Answer 6

AUSS
MRCP

Question 7

Management of PBC

Answer 7

First-line: ursodeoxycholic acid

Pruritus: cholestyramine

fat-soluble vitamin supplementation

liver transplantation
e.g. if bilirubin > 100

Question 8

Complications of PBC

Answer 8

cirrhosis → portal hypertension → ascites, variceal haemorrhage

osteomalacia and osteoporosis

hepatocellular carcinoma