Primary sclerosing cholangitis

Question 1

Define primary sclerosing cholangitis (PSC)

Answer 1

Primary sclerosing cholangitis = chronic cholestatic liver disease characterized by progressive inflammatory fibrosis, obliteration and stricturing of intrahepatic and extrahepatic bile ducts

Question 2

What is the aetiology of PSC?

Answer 2

Unknown – suspected multifactorial
o Immune, genetic and toxic/infective triggers suggested

In IBD, certain organisms are able to traverse the bowel wall

Question 3

What are the risk factors for PSC?

Answer 3

IBD (Especially UC)
o 75-90% with PSC have IBC and 87% of these are UC
o 70% with PSC have UC
o 5% with UC go on to get PSC

FH and genetics
o HLA-A1, HLA-B8, HLA-DR3

HIV

Question 4

What is the epidemiology of PSC?

Answer 4

• Prevalence of 2-7 in 100,000
• Usually presents age 25-40 y/o
• 70% are men

Question 5

What are the symptoms of PSC?

Answer 5

Asymptomatic

Intermittent episodes
o	RUQ pain
o	Jaundice
o	Pruritus
o	Weight loss
o	Fatigue

Episodes of
o Fever
o Rigors

UC symptoms

Question 6

What are the signs of PSC?

Answer 6

• Jaundice
• Hepatosplenomegaly
• Spider naevi
• Palmar erythema
• Ascites
• Weight loss

Question 7

What are the investigations for PSC?

Answer 7

Laboratory testing
o	Bloods - LFTs: raised ALP, GGT, ALT, AST, bilirubin and decreased albumin (late stage)
o	Serology:
Increased IgM
P-ANCA (antibodies) (70%)
aCL antibodies
ANA antibodies (30%)

Cholangiography
o ERCP - Stricturing and interspersed dilation/beading of intrahepatic bile ducts, Small diverticula on common bile duct
o MRCP = Non-invasive imaging of biliary tree = preferred, DIAGNOSTIC