Primary biliary cholangitis (PBC)

Question 1

1) What is PBC?
2) This causes obstruction of the outflow of bile - what is this known as?
3) What does the back-pressure of the bile obstruction and the overall disease process result in?

Answer 1

1) Condition where the immune system attacks the small bile ducts (interlobular bile ducts) within the liver
2) Cholestasis
3) Liver cirrhosis

Question 2

1) Name 2 things that are usually excreted through the bile ducts into the intestines
2) What does an obstruction to the outflow of these chemicals cause?

Answer 2

1) Bile acids, cholesterol and bilirubin
2) Buildup of these chemicals in the blood

Question 3

1) What does the accumulation of bile acids in the blood cause?
2) What does the the accumulation of bilirubin in the blood cause?
3) Name 1 thing the accumulation of cholesterol in the blood causes
4) Name 1 thing the lack of bile acids in stools causes
5) What effect is seen as a result of a lack of bilirubin in stools?

Answer 3

1) Itchy skin
2) Jaundice
3) Cholesterol deposits in the eyes (xanthelasma) and in blood vessels which increases cardiovascular disease risk
4) Gastrointestinal disturbance, malabsorption of fats and greasy stools
5) Pale stools

Question 4

1) What is the hallmark of PBC?
2) What are the 2 things that contribute to cause PBC
3) Name 2 risk factors for developing PBC

Answer 4

1) Antimitochondrial antibodies and granulomas
2) Unknown environmental triggers, genetic predisposition
3) Smoking, family history, many UTIs, history of other autoimmune diseases

Question 5

Name 3 ways a patient may present

Answer 5

• Fatigue
• Pruritus
• GI disturbance and abdominal pain
• Jaundice
• Pale stools
• Xanthoma and xanthelasma
• Signs of cirrhosis and failure

Question 6

Name 2 complications of PBC

Answer 6

• Cirrhosis complications
• Osteoporosis
• Hepatocellular carcinoma
• Fat soluble vitamins deficiency (due to malabsorption) which can lead to coagulopathy and osteomalacia

Question 7

1) What antibody is most specific to PBC?
2) Name another marker than may be raised
3) What is used in diagnosing and staging the disease?

Answer 7

1) Anti-mitochondrial antibodies
2) ESR, IgE, ALP
3) Liver biopsy

Question 8

1) What can be given to reduce the intestinal absorption of cholesterol?
2) Cholestyramine is a bile acid sequestrant in that it binds to bile acids to prevent absorption in the gut - what symptom can this help with?
3) Name another aspect of the management of PBC

Answer 8

1) Ursodeoxycholic acid
2) Pruritus
3) Prophylaxis with fat soluble vitamins, codeine phosphate for diarrhoea, osteoporosis prevention