Primary Biliary Cholangitis

Question 1

What is primary biliary cholangitis?

Answer 1

autoimmune condition where the immune system attacks the small bile ducts in the liver, resulting in obstructive jaundice and liver disease.

Question 2

What ducts are first affected in primary biliary cholangitis?

Answer 2

Intralobar ducts (canals of herring)

Question 3

Where does inflammation occur in primary biliary cholangitis?

Answer 3

Small bile ducts
Epithelial cells

Question 4

What 3 things are excreted through the bile ducts into the intestines?

Answer 4

Bile acid
Bilirubin
Cholesterol

Question 5

What symptom does a rise in bile acids cause?

Answer 5

Pruritis (itching)

Question 6

What symptom does a rise in bilirubin cause?

Answer 6

Jaundice

Question 7

What clinical sign is seen with a rise in cholesterol?

Answer 7

Xanthelasma

Question 8

What is the function of bile acids?

Answer 8

Digestion of fats

Question 9

What happens when there is reduced or absent bile acids?

Answer 9

Malabsorption of fat
Greasy stools

Question 10

What 2 symptoms are caused by a lack of bilirubin being excreted?

Answer 10

Pale stools
Dark urine

Question 11

What is the typical patient with primary biliary cholangitis?

Answer 11

White woman aged 40-60 with another autoimmune/rheumataogical disease

Question 12

What are the presenting features of primary biliary cholangitis? (6)

Answer 12

• Fatigue
• Pruritus (itching)
• Gastrointestinal symptoms and abdominal pain
• Jaundice
• Pale, greasy stools
• Dark urine

Question 13

What can be seen on examination in primary biliary cholangitis? (4)

Answer 13

○ Xanthoma and xanthelasma (cholesterol deposits)
○ Excoriations (scratches on the skin due to itching)
○ Hepatomegaly
○ Signs of liver cirrhosis and portal hypertension in end-stage disease (e.g., splenomegaly and ascites)

Question 14

What is seen on LFTs in primary biliary cholangitis?

Answer 14

Raised ALP
Bilirubin raised later in disease

Question 15

What autoantibodies are found in primary biliary cholangitis? (2)

Answer 15

○ Anti-mitochondrial antibodies (AMA) are the most specific to PBC and form part of the diagnostic criteria
○ Anti-nuclear antibodies are present in about 35% of patients - not very specific

Question 16

What immunoglobulin is raised in primary biliary cholangitis?

Answer 16

IgM

Question 17

What investigation is used in diagnosing and staging primary biliary cholangitis?

Answer 17

Liver biopsy

Question 18

What is the first line treatment in primary biliary cholangitis? How does it work?

Answer 18

Ursodeoxycholic acid

It is a non-toxic, hydrophilic bile acid that protects the cholangiocytes from inflammation and damage.
It makes the bile less harmful to the epithelial cells of the bile ducts.
Reduces intestinal absorption of cholesterol
It slows the disease progression and improves outcomes.

Question 19

What drug is used as second line treatment of primary biliary cholangitis?

Answer 19

Obeticholic acid

Question 20

What drug is used for symptoms of Pruritis?

Answer 20

Colestyramine

Question 21

What are the complications of primary biliary cholangitis? (7)

Answer 21

Liver cirrhosis -> HCC
Fat-soluble vitamin deficiency (A, D, E and K)
Osteoporosis
Hyperlipidaemia (raised cholesterol)
Sjögren’s syndrome (dry eyes, dry mouth and vaginal dryness)
Connective tissue diseases (e.g., systemic sclerosis)
Thyroid disease