Primary Sclerosing Cholangitis

Question 1

Definition

Answer 1

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

Question 2

Pathogenesis

Answer 2

o Periductal inflammation with periductal concentric fibrosis
o Portal oedema
o Bile duct proliferation
o Expansion of portal tracts
o Progressive fibrosis
o Development of biliary cirrhosis

Question 3

Aetiology

Answer 3

• UNKNOWN
• Possible immune and genetic predisposition with environmental triggers
• Close association with inflammatory bowel disease (especially ulcerative colitis)
• IMPORTANT: ulcerative colitis is present in about 70% of patients with PSC

Question 4

Epidemiology

Answer 4

• Usually presents between 25-40 years of age

Question 5

Presenting symptoms

Answer 5

• May be asymptomatic and diagnosed after persistently raised ALP

• May present with:
o Intermittent jaundice
o Pruritis
o RUQ pain
o Weight loss
o Fatigue

• May present with episodes of fever and rigors caused by acute cholangitis (but this is
less common)

• IMPORTANT: check for a history of ulcerative colitis

Question 6

Signs on physical examination

Answer 6

• May have no signs
• Jaundice
• Hepatosplenomegaly
• Spider naevi
• Palmar erythema
• Ascites

Question 7

Investigations (bloods)

Answer 7

o LFTs:
• High ALP + GGT
• Mildly elevated ALT + AST
• Low albumin + high bilirubin (later stages)

Question 8

Investigations (serology)

Answer 8

o IgG high in children
o IgM high in adults
o ASMA and ANA present in 30%
o Anti-mitochondrial antibodies (AMA) usually ABSENT
o Perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) present in 70%

Question 9

Investigations

Answer 9

• ERCP
o Shows stricturing and interspersed dilation of intrahepatic and (occasionally)
extraheptic bile ducts
o Small diverticuli on the common bile duct may be seen

• MRCP
o Enables non-invasive imaging of the biliary tree

• Liver Biopsy
o Confirm diagnosis and allows staging