Primary Biliary Cirrhosis

Question 1

What is primary biliary cirrhosis. (2)

Answer 1

Chronic inflammation and destruction of the small and medium bile ducts.
It is a chronic, progressive cholestatic liver disease.

Question 2

Who gets affected by primary biliary cirrhosis. (2)

Answer 2

80-90% women.

Age 30-60.

Question 3

What are the clinical features of primary biliary cirrhosis. (9)

Answer 3

Fatigue. 
Pruritus. 
Arthralgia. 
Xanthelasma. 
Hepatomegaly. 
Splenomegaly. 
Jaundice (rare). 
Signs of chronic liver disease. 
Osteoporosis.

Question 4

What is the antibody strongly associated with PBC.

Answer 4

AMA (antimitochondrial antibodies).

Question 5

What is the characteristic histological finding in PBC.

Answer 5

Granulomatous inflammation of the portal tracts, leading to progressive damage and eventually loss of the small and middle sized bile ducts.

Question 6

What happens in PBC after the damage of the bile ducts. (3)

Answer 6

Fibrosis, which in turn leads to cirrhosis of the liver, and portal hypertension.

Question 7

What does PBC initially present with. (2)

Answer 7

Itching.

And possible tiredness.

Question 8

What sort of onset does PBC have.

Answer 8

Insidious.

Question 9

What parts of the world is PBC most common. (2)

Answer 9

Europe.

North America.

Question 10

Where in the world is PBC rare. (2)

Answer 10

Africa.

Asia.

Question 11

PBC is more common amongst _________

Answer 11

Smokers.

Question 12

What other conditions is PBC associated with. (4)

Answer 12

Thyroid disease (hypothyroidism).
Sicca syndrome.
Systemic sclerosis.
Coeliac disease.

Question 13

What gene is associated with PBC.

Answer 13

HLA-DR8

Question 14

What does AMA target.

Answer 14

Pyruvate dehydrogenase complex.

Question 15

What is the osteomalacia associated with PBC caused by.

Answer 15

Malabsorption of the fat soluble vitamins.

Question 16

What is the osteoporosis associated with PBC caused by.

Answer 16

Hepatic osteodystrophy.

Question 17

What is the prevalence of PBC.

Answer 17

4/ 100,000

Question 18

What may be present on routine blood tests in a patient with undiagnosed PBC. (2)

Answer 18

AMA.

Raised serum alkaline phosphatase.

Question 19

What is seen in 70% of patients with PBC.

Answer 19

Keratoconjunctivitis sicca.

Question 20

What is keratoconjunctivitis sicca.

Answer 20

Dry eyes and mouth.

Question 21

What type of condition is PBC.

Answer 21

Autoimmune.

Question 22

What risk factors increase the likelihood of developing PBC. (6)

Answer 22

Family history (seen in 1-6%).
Chronic UTIs. 
Smoking. 
Past pregnancy. 
Other autoimmune diseases. 
Use of nail polish/hair dye.

Question 23

What are the fat soluble vitamins. (4)

Answer 23

A.
D.
E.
K.

Question 24

Why is there malabsorption of fat soluble vitamins in PBC.

Answer 24

Due to cholestasis.

Question 25

What are the complications of PBC. (5)

Answer 25

Those associated with cirrhosis. Osteoporosis. Malabsorption of fat soluble vitamins resulting from cholestasis causes osteomalacia. Decreased bilirubin in the gut lumen (along with the cholestasis) results in coagulopathy. Hepatocellular carcinoma.