Primary Biliary Cholangitis

Question 1

What is Primary Biliary Cirrhosis?

Answer 1

Intralobular bile ducts become damaged by an autoimmune chronic inflammatory process

Question 2

What does Primary Biliary Cirrhosis lead to?

Answer 2

Progressive cholestasis which will eventually lead to cirrhosis

Question 3

What type of condition is Primary Biliary Cirrhosis?

Answer 3

Autoimmune condition

Question 4

What is cholestasis?

Answer 4

Obstruction of the outflow of bile

Question 5

What is the pathophysiology of Primary Biliary Cirrhosis?

Answer 5

Bile acids, Bilirubin and Cholesterol are usually excreted into intestine via bile ducts
If bile ducts are blocked- chemicals go into blood

Question 6

What are the symptoms of primary biliary cholangitis?

Answer 6

Itching (bile acids)
Jaundice (bilirubin)
Xanthelasmata (cholesterol deposits on the skin)
RUQ pain 
Hyperpigmentation over pressure points

Question 7

How is primary biliary cholangitis diagnosed?

Answer 7

• Anti-mitochondrial antibodies

* Raised serum IgM

Question 8

What is Primary Biliary Cholangitis associated with?

Answer 8

• Sjogren’s syndrome (80% patients)
• rheumatoid arthritis
• systemic sclerosis
• thyroid disease

Question 9

What is the first line management of Primary Biliary Cholangitis?

Answer 9

Ursodeoxycholic acid

Question 10

What is the management of pruritis?

Answer 10

Cholestyramine

Question 11

What is the function of Ursodeoxycholic acid?

Answer 11

Slows disease progression

Improves symptoms