Primary Biliary vs Primary Sclerosing Cholangitis Flashcards

1
Q

What is Primary Biliary Cholangitis

A

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

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2
Q

What is Primary Sclerosing Cholangitis

A

Intra and extrahepatic bile duct inflammation and sclerosis

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

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3
Q

Epidemiology of Primary Biliary Cholangitis

A

40-60 mainly

Women X9 risk

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4
Q

Epidemiology of Primary Sclerosing Cholangitis

A

40

Men X2 risk

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5
Q

Clinical signs and symptoms of Primary Biliary Cholangitis

A
Fatigue
Pruritus
Xanthelasma
Discomfort over liver
Blotchiness of hands
Pale stools
Nausea
Go off alcohol/cigarettes
Hepatomegaly
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6
Q

What is Pruritus

A

Severe itching of the skin

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7
Q

What is Xanthelasma

A

Yellow eye patches

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8
Q

Clinical signs and symptoms of Primary Sclerosing Cholangitis

A

Fatigue
Pruritus
Jaundice

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9
Q

Investigations of Primary Biliary Cholangitis

A

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

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10
Q

Investigations of Primary Sclerosing Cholangitis

A

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

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11
Q

Treatment of Primary Biliary Cholangitis

A
  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
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12
Q

Treatment of Primary Sclerosing Cholangitis

A
  1. Avoid alcohol
  2. Itch: Cholestyramine and Vitamin supplement
  3. Transplant
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13
Q

Associations and Complications of Primary Biliary Cholangitis

A

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

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14
Q

Associations and Complications of Primary Sclerosing Cholangitis

A

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

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15
Q

Raised globulins of PBC and PSC and AIH (hepatitis)

A

PBC - IgM
PSC - Variable
AIH - IgG

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16
Q

Autoantibodies of PBC and PSC

A

PBC - Antimitochondrial

PSC - Antineutrophil cytoplasmic (ANCA - not specific)

17
Q

What can PSC be a complication of?

A

Cholangiocarcinoma
Liver cirrhosis
(Correlation with UC)

18
Q

Cause of obstruction in PBC vs PSC

A

PBC - granulomatous inflammation destroying bile ducts

PSC - fibrosis destroying bile ducts

19
Q

Key microscopic feature of PBC vs PSC

A

PBC - florid duct lesion (granulomas)

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