Primary Biliary vs Primary Sclerosing Cholangitis

Question 1

What is Primary Biliary Cholangitis

Answer 1

Autoimmune disorder against hepatocytes
Causes biliary duct degeneration and granuloma.
Intrahepatic small bile duct destruction

Question 2

What is Primary Sclerosing Cholangitis

Answer 2

Intra and extrahepatic bile duct inflammation and sclerosis

Cholestatic liver disease characterised by fibrosing inflammatory destruction of both intra and extrahepatic bile ducts

Question 3

Epidemiology of Primary Biliary Cholangitis

Answer 3

40-60 mainly

Women X9 risk

Question 4

Epidemiology of Primary Sclerosing Cholangitis

Answer 4

40

Men X2 risk

Question 5

Clinical signs and symptoms of Primary Biliary Cholangitis

Answer 5

Fatigue
Pruritus
Xanthelasma
Discomfort over liver
Blotchiness of hands
Pale stools
Nausea
Go off alcohol/cigarettes
Hepatomegaly

Question 6

What is Pruritus

Answer 6

Severe itching of the skin

Question 7

What is Xanthelasma

Answer 7

Yellow eye patches

Question 8

Clinical signs and symptoms of Primary Sclerosing Cholangitis

Answer 8

Fatigue
Pruritus
Jaundice

Question 9

Investigations of Primary Biliary Cholangitis

Answer 9

+AMA (Anti-mitochondrial antibody - in blood)
Raised IgM
Abnormal LFTs, USS (ultrasound scan), MRI, Biopsy

Question 10

Investigations of Primary Sclerosing Cholangitis

Answer 10

MRCP or endoscopic retrograde cholangiography
ANCA (Anti-neutrophil cytoplasmic antibody)
Abnormal LFTs, USS, MRI, liver biopsy

Question 11

Treatment of Primary Biliary Cholangitis

Answer 11

1. Lower alcohol
2. Itch: Cholestyramine OR antihistamines, opiate antagonists, rifampicin, UV, plasmapheresis
3. Fatigue: Modafinil, Sertraline
4. Disease modifier: Ursodeoxycholic acid, Methotrexate
5. Transplant

Question 12

Treatment of Primary Sclerosing Cholangitis

Answer 12

1. Avoid alcohol
2. Itch: Cholestyramine and Vitamin supplement
3. Transplant

Question 13

Associations and Complications of Primary Biliary Cholangitis

Answer 13

Associations: Other autoimmune e.g. RA, Sjorgen’s
Complications: Cirrhosis, Liver failure, Osteoporosis, Increased risk of HCC (Hepatocellular carcinoma)

Question 14

Associations and Complications of Primary Sclerosing Cholangitis

Answer 14

Associations: IBD, especially Ulcerative Colitis (80%)
Complications: Liver failure, cirrhosis, Vit ADEK deficiency, increased risk of cholangiocarcinoma

Question 15

Raised globulins of PBC and PSC and AIH (hepatitis)

Answer 15

PBC - IgM
PSC - Variable
AIH - IgG

Question 16

Autoantibodies of PBC and PSC

Answer 16

PBC - Antimitochondrial

PSC - Antineutrophil cytoplasmic (ANCA - not specific)

Question 17

What can PSC be a complication of?

Answer 17

Cholangiocarcinoma
Liver cirrhosis
(Correlation with UC)

Question 18

Cause of obstruction in PBC vs PSC

Answer 18

PBC - granulomatous inflammation destroying bile ducts

PSC - fibrosis destroying bile ducts

Question 19

Key microscopic feature of PBC vs PSC

Answer 19

PBC - florid duct lesion (granulomas)

PSC - concentric ‘onion-skin’ fibrosis around bile ducts