Primary sclerosing cholangitis

Primary sclerosing cholangitis is a rare disease of unknown aetiology characterised by chronic inflammation and fibrosis of the bile duct.

Narrowing of the bile duct lumen usually occurs throughout the biliary tree. Less commonly, changes are confined to the intra-hepatic or extra-hepatic ducts but is seldom short enough to resemble a post-traumatic or focal malignant stricture.

• there is a strong association with inflammatory bowel disease (75 per cent of cases), particularly ulcerative colitis but also Crohn’s disease
• in addition to complications related to end-stage liver disease, patients may develop a number of other complications e.g. biliary strictures, infective cholangitis, cholangiocarcinoma
• majority of patients will be asymptomatic at the time of diagnosis, although they may have advanced disease

The diagnosis of primary sclerosing cholangitis should be suspected in a patient with ulcerative colitis who has abnormal LFTs, especially a raised alkaline phosphatase

• pruritus and fatigue are early symptoms - patients may also present with fevers, night sweats and right upper quadrant pain (1)

Reference:

1. Prescriber 2003; 14(23):20-9.