Primary sclerosing cholangitis

Question 1

What happens in PSC?

Answer 1

Where the intrahepatic or extrahepatic ducts become strictured and fibrotic.

This causes an obstruction to the flow of bile out of the liver

Chronic obstruction leads eventually to liver inflammation (hepatitis), fibrosis and cirrhosis

Question 2

What causes PSC?

Answer 2

The cause is mostly unclear although there is likely to be a combination of genetic, autoimmune, intestinal microbiome and environmental factors

Association with UC - 70% of PSC is in UC patients

Question 3

Risk factors for PSC

Answer 3

UC
Male
Aged 30-40
Family history

Question 4

Presentation of PSC

Answer 4

Jaundice
Chronic right upper quadrant pain
Pruritus
Fatigue
Hepatomegaly

Question 5

Investigations in PSC suspected

Answer 5

LFTs:

• ALP most deranged (cholestatic picture)
• Bilirubin may rise as strictures becomes more severe preventing bilirubin from being excreted through the bile duct.
• AST/ALT can become deranged if it progresses to cause hepatitis

GOLD STANDARD TEST is MRCP (to look for strictures and lesions in bile duct)

Autoantibodies aren’t helpful for diagnosis, but can indicate where there is an autoimmune element to the disease that may respond to immunosuppression.

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

Question 6

Complications and associations in PSC

Answer 6

Acute cholangitis

Cholangiocarcinoma develops in 10-20% of cases

Cirrhosis and liver failure

Biliary strictures

Colorectal cancer

Fat soluble vitamin deficiencies (bile can’t help with fat digestion and absorption)

Question 7

Management of PSC

Answer 7

ERCP - dilate and stent strictures

Ursodeoxycholic acid can slow progression

Colestyramine - binds to bile acid and reduces pruritits

Monitoring for complications e.g. cholangiocarcinoma, cirrhosis, varices

Only curative option is liver transplant - but has its own problems