Autoimmune liver diseases

Question 1

PBC - definition

Answer 1

Damage to the bile ducts in the liver

Affects the small bile ducts

Question 2

PBC - aetiology

Answer 2

Middle aged women

Background of fatty liver disease

Question 3

PBC - clinical presentation

Answer 3

Asymptomatic
Fatigue 
Jaundice
Itch without rash
Xanthelasma

Question 4

PBC - investigations

Answer 4

+AMA

Cholestatic LFT’s (increased Alk phos, GGT)

Question 5

PBC - management

Answer 5

Urseodeoxycholic acid - to change composition of bile and make it flow easier
Liver transplant
IMMUNOSUPPRESSANTS DONT WORK

Question 6

PBC - pathology

Answer 6

Granulomas form around duct
Neutrophils
Bile duct loss
Chronic portal inflammation

Question 7

PBC - disease progression

Answer 7

Bile duct loss
Cholestasis
Liver injury
Inflammation
Fibrosis
Cirrhosis

Question 8

PSC - definition

Answer 8

Damage to the bile ducts in the liver

Affects the medium and large bile ducts

Question 9

PSC - aetiology

Answer 9

Males

UC

Question 10

PSC - clinical presentation

Answer 10

Asymptomatic 
Jaundice 
Abdominal pain
Fatigue
Itch
Rigors 
Hepatomegaly

Question 11

PSC - investigations

Answer 11

Cholestatic LFTs
MRCP
Liver biopsy

Question 12

PSC - management

Answer 12

Transplant

Question 13

PSC - pathology

Answer 13

Onion skinning fibrosis around the bile ducts

Question 14

Autoimmune hepatitis - aetiology

Answer 14

Young women

Chronic hepatitis

Question 15

Autoimmune hepatitis - types

Answer 15

TYPE 1

• more common
• affects adults
• extrahepatic manifestations
• ASMA, ANA

TYPE 2

• less common
• children
• AMA

Question 16

Autoimmune hepatitis - clinical presentation

Answer 16

Jaundice
Abdominal pain
Hepatomegaly
Splenomegaly
Malaise
Fatigue
Nausea
Weight loss

Question 17

Autoimmune hepatitis - investigations

Answer 17

+ASMA
Hepatic LFT’s - increase ALT, AST
Liver biopsy

Question 18

Autoimmune hepatitis - management

Answer 18

Corticosteroids (prednisolone)

Immunosuppressants (azathioprine)