Primary sclerosing cholangitis

Question 1

What is sclerosing cholangitis?

Answer 1

Choelstatic liver disease resulting from inflammation of the biliary tree (intra/extrahepatic bile ducts) leading to scarring and lumen obliteration, cirrhosis and end stage liver disease.

Question 2

What is the aetiology of primary sclerosing cholangitis?

Answer 2

-A/w IBD (more common UC); up to 70% of pts

Question 3

What is the aetiology of secondary sclerosing cholangitis?

Answer 3

• long term choledocholithiasis
• cholangiocarcinoma
• surgical trauma
• contiguous inflammatory process
• post ERCP
• HIV/AIDS (HIV cholangiopathy)

Question 4

Signs and symptoms of sclerosing cholangitis?

Answer 4

• Insidious
• May present w/ fatigue/pruritus
• signs of episodic bacterial cholangitis secondary to biliary obstruction

Question 5

Diagnostic investigations of sclerosing cholangitis?

Answer 5

• Increased ALP
• Mildly increased AST
• p-ANCA (30-80%)
• Elevated IgM (40-50%)
• ERCP narrowing + beading of ducts

Question 6

If ERCP demonstrates narrowing of intrahepatic ducts only, what should be investigated?

Answer 6

Anti-mitochondrial antibody to rule out primary biliary cirrhosis.

Question 7

What are the complications of sclerosing cholangitis?

Answer 7

• Repeated bouts of cholangitis > complete biliary obstruction > secondary biliary cirrhosis and hepatic failure
• Increased incidence of cholangiocarcinoma (10-15%)

Question 8

Management of sclerosing cholangitis?

Answer 8

• Annual MRCP (cholangiocarcinoma screening)
• Endoscopic sphincterotomy, biliary stent in dominant CBD stricture
• ABx for cholangitis
• Emergency drainage of suppurative CBD
• Liver transplant best treatment

Question 9

Prognosis of sclerosing cholangitis?

Answer 9

Poor. Mean survival after diagnosis is 4 - 10y.

Question 10

Epidemiology of PSC?

Answer 10

• 2M:1F
• Generally 40s - 50s
• ~2/3s have IBD

Question 11

RFx for PSC?

Answer 11

• Malge gender
• IBD
• Genetic predisposition (1st’ relatives)