Primary Sclerosing Cholangitis

Question 1

Define

Answer 1

Progressive chronic cholestasis with bile duct inflammation and strictures
→ Characterised by progressive inflammatory fibres and obliteration of the intrahepatic and extrahepatic bile ducts

Question 2

Causes

Answer 2

• Periductal inflammation with periductal concentric fibrosis (onionskin), portal oedema, bile duct proliferation and expansion of portal tracts, progressive fibrosis and development of biliary cirrhosis

Causes:

• 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 3

Risk factors

Answer 3

Male sex

• HLA-A1; B8l DR3
• AI hep
• IBD (usually UC)

Question 4

Symptoms

Answer 4

May be asymptomatic and diagnosed after persistently raised ALP

May present with:

• Intermittent jaundice
• Pruritis
• RUQ pain
• Weight loss
• 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 5

Signs

Answer 5

May have no signs

Jaundice

Hepatosplenomegaly

Spider naevi

Palmar erythema

Ascites

Question 6

Investigations

Answer 6

Bloods

LFTs:

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

Serology

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

ERCP

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

MRCP

• Enables non-invasive imaging of the biliary tree

Liver Biopsy

Confirm diagnosis and allows staging