Primary billiary cirrhosis

Question 1

What is the definition of ‘Primary biliary cirrhosis’

Answer 1

1. chronic progressive liver disease
2. autoimmune origin
3. destruction of the intralobular bile ducts

Question 2

What is the first presenting feature?

Answer 2

1. Patients are usually asymptomatic

2. Fatigue is the first symptom

Question 3

Name 4 clinical features in advanced disease?

Answer 3

1. Pruritus
2. Hepatosplenomegaly
3. Abdominal pain
4. Jaundice

Question 4

What condition is Primary Billiary cirrhosis associated with?

Answer 4

1. Coeliacs’s
2. Sjorens
3. Autoimmune thyroiditis

Question 5

Which LFTs are raised?

Answer 5

1. ALP raised

2. GGT raised

Question 6

Which characteristic antibody is found?

Answer 6

anti mitochondrial antibody

Question 7

Describe the results of blood tests in Primary billary cirrhosis?

Answer 7

1. ALP raised
2. GGT raised
3. IgM and Anti mitochondrial antibodies

Question 8

What is the main tx?

Answer 8

1. Ursodeoxycholic acid

Question 9

Treatment adjuncts in primary biliary cirrhosis?

Answer 9

1. Cholestyramine - treats pruritus

2. Vitamin supplementation