Primary Biliary Cholangitis

Question 1

What is PBC?

Answer 1

Primary biliary cholangitis

A chronic liver disorder

Question 2

What is the pathophysiology of PBC?

Answer 2

Interlobular bile ducts are damaged by chronic granulomatous inflammation which causes progressive cholestasis which leads to cirrhosis

Question 3

What conditions are associated with PBC?

Answer 3

Thyroid disease
RA
Sjogren’s syndrome
Systemic sclerosis

Question 4

What are the risk factors for PBC?

Answer 4

Female
40-60 years
FHx of autoimmune disease
Personal history of autoimmune disease

Question 5

What are the features of PBC?

Answer 5

Often asymptomatic

Fatigue 
Pruritus 
Jaundice 
Hyperpigmentation 
Arthralgia

Question 6

What investigations are done in suspected PBC?

Answer 6

Bloods

• LFTs
• clotting screen
• TFTs
• lipids

AMA antibodies

USS of liver

Question 7

What do the LFTs show in PBC?

Answer 7

Raised ALP
Raised ALT
Raised GGT

Question 8

How is the pruritus in PBC managed?

Answer 8

Chostyramine

Question 9

How is PBC managed medically?

Answer 9

Cholestryamine
Fat-soluble vitamin supplements
Ursodeoxycholic acid

Question 10

What is surgical management of PBC?

Answer 10

Liver transplant

Question 11

When is liver transplant indicated in PBC?

Answer 11

Bilirubin >100

Question 12

What are the complications of PBC?

Answer 12

Cirrhosis
Osteomalacia/osteoporosis
Increased risk of HCC