Primary biliary cirrhosis/primary sclerosing cholangitis

Question 1

Pathophysiology of primary biliary cirrhosis?

Answer 1

Chronic granulomatous inflammation of interlobular bile ducts

Question 2

How does PBC present? (4)

Answer 2

May be asymptomatic raised Alk Phos on LFTs
Lethargy
Itching
Jaundice

Question 3

What antibody is associated with PBC?

Answer 3

AMA M2- highly specific

Question 4

Disease associations? (4)

Answer 4

Sjogren’s syndrome
RA
Systemic sclerosis
Thyroid disease

Question 5

Investigation of suspected PBC:

a) blood/serology
b) imaging

Answer 5

a) elevated Alk Phos and GGT; autoantibodies

b) USS liver

Question 6

Management of PBC (5)

Answer 6

Cholestyramine (itch)
Codeine phosphate (diarrhoea)
Fat soluble vitamin supplements
Ursodeoxycholic acid
Transplantation

Question 7

What is primary sclerosing cholangitis characterized by pathologically?

Answer 7

Inflammation, fibrosis and stricture of the extra and intra hepatic bile ducts

Question 8

Risk of what cancer is increased in PSC?

Answer 8

Cholangiocarcinoma

Question 9

Disease associations of PSC? (3)

Answer 9

Ulcerative colitis
Crohn’s disease (rarely)
HIV

Question 10

Diagnostic test for PSC?

Answer 10

MRCP

Question 11

Symptoms of PSC? (4)

Answer 11

Jaundice, pruritus, abdominal pain, fatigue

Question 12

Classical skin sign of PBC?

Answer 12

Xanthelasma of the eyelid