Autoimmune liver disease

Question 1

what are the 3 types of autoimmune hepatitis?

Answer 1

type 1: anti-nuclear antibody (ANA)/ anti-smooth muscle antibodies (SMA)
- affects both children and adults

type 2: anti-liver/kidney microsomal type 1 antibodies (LKM1)
- only affects children

type 3: soluble liver/kidney antigen (SLA)
- affects middle aged adults

Question 2

what are features of autoimmune hepatitis?

Answer 2

may present with signs of chronic liver disease
acute hepatitis: jaundice, pyrexia, RUQ pain (uncommon)
ammenorrhoea

ANA/SMA/LKM antibodies
raised IgG levels

Question 3

how is autoimmune hepatitis diagnosed?

Answer 3

liver biopsy

• inflammation beyond the limiting plate ‘piecemeal necorosis’ and bridging necrosis

Question 4

what is the management for autoimmune hepatitis?

Answer 4

steroids
+ immunosuppressant i.e. Azathioprine

liver transplantation

Question 5

what is the management for primary biliary cholangitis?

Answer 5

ursodeoxycholic acid

Question 6

what is the management for primary sclerosing cholangitis?

Answer 6

stenting

liver transplant

Question 7

what is the difference in the pathophysiology of PSC and PBC?

Answer 7

PBC: granulomatou sinfmmation in the extrahepatic bile ducts

PSC: fibrosis/stricturing destroying bile ducts, intra and extra hepatic bile ducts