Primary Sclerosing Cholangitis

Question 1

What is primary sclerosing cholangitis?

Answer 1

Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic liver disease, characterised by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts, resulting in diffuse, multi-focal stricture formation.

Question 2

What are the risk factors for primary sclerosing cholangitis?

Answer 2

> Male
IBD
Genetic predisposition

Question 3

What are the signs and symptoms of primary sclerosing cholangitis?

Answer 3

> Abdo pain
> Pruritis
> Fatigue
> Weight loss
> Fever
> jaundice
> Steatorrhoea
> Splenomegaly
> Ascites
> Encephalopathy

Question 4

What is the epidemiology of primary sclerosing cholangitis?

Answer 4

Prevalence:
5.58 per 100,000 in the UK
(relatively rare)

Question 5

What investigations would you do for primary sclerosing cholangitis?

Answer 5

> serum alkaline phosphatase
> serum gamma-GT
> serum aminotransferases (aspartate aminotransferase, alanine aminotransferase)
> serum total bilirubin
> serum albumin
> FBC
> prothrombin time
> atypical antineutrophil cytoplasmic auto-antibody (ANCA)
> antimitochondrial antibody
> abdominal ultrasound
> MRCP
> ERCP