Primary Biliary Cirrhosis

Question 1

Definition

Answer 1

A chronic inflammatory liver disease involving progressive destruction of intrahepatic bile ducts, leading to cholestasis, and, ultimately, cirrhosis

Question 2

Aetiology

Answer 2

• UNKNOWN
• Likely to be autoimmune
• Genetic and environmental factors are involved
• An environmental trigger may cause bile duct epithelial injury, which then leads to a Tcell mediated autoimmune response directed against bile duct epithelial cells

Question 3

Epidemiology

Answer 3

Mainly affects middle-aged women (9:1 female: male)

Question 4

Presenting symptoms

Answer 4

• May be an incidental finding on blood tests

• Insidious onset with vague symptoms such as:
o Fatigue
o Weight loss
o Pruritis

• Rarely, it may cause discomfort in the RUQ

• May present with a complication of liver decompensation (e.g. jaundice, ascites,
variceal haemorrhage)

• May present with symptoms of associated conditions (e.g. Sjogren’s syndrome, arthritis, Raynaud’s phenomenon)

Question 5

Signs on physical examination

Answer 5

• Early - may be no signs

• Late:
o Jaundice
o Skin pigmentation
o Scratch marks
o Xanthomas (secondary to hypercholesterolaemia)
o Hepatomegaly
o Ascites
o Signs of chronic liver disease

Question 6

Investigations (bloods)

Answer 6

o LFT:
• High ALP + GGT
• Bilirubin may be high or normal
• ALT and AST are normal initially but will increase as the disease progresses
and cirrhosis develops

o Clotting: prolongation of PT

o Typical features of PBC:
• Antimitochondrial antibodies (typical feature of PBC)
• High IgM
• High Cholesterol

o TFTs - because PBC is associated with autoimmune thyroid disease

Question 7

Investigations (other)

Answer 7

• Ultrasound
o Exclude extrahepatic biliary obstruction (e.g. gallstones)

• Liver Biopsy
o In PBC, it will show chronic inflammatory cells and granulomas around the intrahepatic bile ducts, destruction of bile ducts, fibrosis and regenerating
nodules of hepatocytes