Primary biliary cirrhosis

Question 1

define primary biliary cirrhosis?

Answer 1

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

Question 2

The what is the cause of primary biliary cirrhosis?

Answer 2

likely to be autoimmune
- bile ducts are damaged by chronic inflammatory process which causes progressive cholestasis which then progresses to be cirrhosis

Question 3

what are the associations of primary biliary cirrhosis?

Answer 3

o Sjogren’s syndrome
o Rheumatoid arthritis
o Systemic sclerosis
o Thyroid disease

Question 4

epidemiology of primary biliary cirrhosis?

Answer 4

• Mainly affects middle-aged women

Question 5

presenting symptoms of primary biliary cirrhosis?

Answer 5

• Insidious onset with vague symptoms such as:
o Fatigue
o Weight loss
o Pruritis
• 10% of patients may have discomfort in the RUQ
• Late presentation can be liver failure signs

Question 6

signs on examination of primary biliary cirrhosis?

Answer 6

•	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 7

what are the typical features of primary biliary cirrhosis on investigation?

Answer 7

• Antimitochondrial antibodies (AMA) subtype M2
• Smooth muscle antibodies in 30% of patients
• High IgM
• High Cholesterol

Question 8

what might the bloods show?

Answer 8

•	High ALP + GGT  
•	Bilirubin may be high or normal 
•	ALT and AST are normal initially but will increase as the disease progresses
o	Clotting: prolongation of PT  
o	TFTs may be deranged

Question 9

what might an USS scan show?

Answer 9

o Exclude extrahepatic biliary obstruction

Question 10

what might a liver biopsy show ?

Answer 10

it will show chronic inflammatory cells and granulomas