Primary sclerosing cholangitis

Question 1

Define primary sclerosing cholangitis (PSC).

Answer 1

A condition in which the intrahepatic or extrahepatic ducts (the biliary tree) become strictured and fibrotic.

Sclerosis= stiffening and hardening of the bile ducts.
Cholangitis = inflammation of the bile ducts.

Question 2

Describe the pathophysiology of PSC.

Answer 2

1. Inflammation of the bile duct
2. Strictures and hardening
3. Obstruction to the flow of bile out of the liver
4. Chronic bile obstruction
5. Leads to liver inflammation
6. Progressive obliterating fibrosis of bile duct branches
7. Cirrhosis
8. Liver failure

Question 3

Give 3 symptoms of PSC.

Answer 3

1. Jaundice
2. Chronic right upper quadrant pain
3. Pruritus (itching)
4. Fatigue
5. Hepatomegaly

75% also have IBD.

Question 4

Give 3 risk factors for PSC.

Answer 4

1. Male
2. FHx
3. Aged 30-40
4. Ulcerative Colitis - 70%

Question 5

With which condition is there an established association to PSC?

Answer 5

Ulcerative colitis
- With around 70% of cases being alongside established ulcerative colitis.

Question 6

Investigations for PSC.

Answer 6

1. LFTs = show a ‘cholestatic’ picture:
   - Alkaline phosphatase (ALP) is the most deranged LFT
   - Rise in bilirubin = as the strictures become more severe
   - Aminotransaminases (I.E. ALT + AST) = deranged particularly as the disease progresses to hepatitis.
2. Autoantibodies
   No antibodies are highly sensitive or specific to PSC. They aren’t very helpful in diagnosis but they can indicate where there is an autoimmune element to the disease that may respond to immunosuppression.

1) Antineutrophil cytoplasmic antibody (p-ANCA) in up to 94%
2) Antinuclear antibodies (ANA) in up to 77%
3) Anticardiolipin antibodies (aCL) in up to 63%

3. Other bloods:
   - U&E
   - FBC
4. Gold standard - MRCP
   - MRI scan of the liver, bile ducts and pancreas
   - May show bile duct lesions or strictures

Question 7

Which 3 autoantibodies can be found for PSC?

Answer 7

1) Antineutrophil cytoplasmic antibody (p-ANCA) in up to 94%
2) Antinuclear antibodies (ANA) in up to 77%
3) Anticardiolipin antibodies (aCL) in up to 63%

Question 8

What is the gold standard test for PSC diagnosis?

Answer 8

MRCP
- Magnetic resonance cholangiopancreatography

Question 9

Management of PSC.

Answer 9

1. Liver transplantation
2. ERCP can be used to dilate and stent any strictures
3. Monitoring for complications
4. Cholestyramine
   - A bile acid sequestrate in that it binds to bile acids to prevent absorption in the gut and can help with pruritus due to raised bile acids

Question 10

Give 3 complications of PSC.

Answer 10

1. Acute bacterial cholangitis
2. Cholangiocarcinoma develops in 10-20% of cases
3. Colorectal cancer
4. Cirrhosis and liver failure
5. Biliary strictures
6. Fat soluble vitamin deficiencies