Primary Sclerosing Cholangitis (PSC)

Question 1

Define:

Answer 1

Chronic cholestatic liver disease characterised by progressive inflammatory fibrosis and obliteration of intrahepatic and extrahepatic bile ducts

Question 2

Aetiology/Risk factors:

Answer 2

UNKOWN.

Thought to have immune and genetic predisposition with environmental trigger.

Strong links to IBD especially UC

Question 3

Epidemiology:

Answer 3

Relatively rare

More common in men than women (2:1)

Presents at 25-40 years

Question 4

Symptoms:

Answer 4

Usually asymptomatic but have consistently raised ALP.

• Intermittent Jaundice
• Pruritus (due to bile salts in the skin)
• Weight loss
• Fatigue
• Fever and Rigor in severe cases due to acute cholangitis infection.
• In later stages symptoms of cirrhosis and acute cholangitits.
• Check for history of UC

Question 5

Signs:

Answer 5

Can have no signs

• Ascites
• Palmar erythema
• Spider naevi
• Hepatosplenomegaly (due to portal hypertension which is caused by the fibrosis pushing on the portal circulation)
• Cirrhosis
• Jaundice

Question 6

Investigations:

Answer 6

Bloods:
-LFT’s - Raised ALT and GGT. Mildly elevated ALP and AST. Later stages - low albumin and high bilirubin.

Liver biopsy - to confirm the diagnosis

ERCP - WILL SHOW A BEADED APPEARANCE.

MRCP

Serology - high IgG in children, high IGM in adults. ASMA and ANA but absence of AMA + pANCA