Treatment

Ursodeoxycholic acid may improve liver function and the patient's symptoms but no definitive improvement in histology or mortality or delay in liver transplant has been demonstrated (1).

Other measures include:

• cholestyramine to relieve pruritus
• correction of deficiencies of fat-soluble vitamins
• percutaneous transhepatic balloon dilatation may be of benefit for dominant strictures

In cases where the disease is largely confined to the distal extrahepatic ducts and the proximal ducts are dilated, a Roux-en-Y hepatico-jejunostomy may be indicated.

Liver transplantation is potentially curative and is indicated where there is hepatic failure, ascites, or oesophageal varices. A 70% five-year survival is reported in major transplant centres but the timing of transplant and patient selection are critical. PSC recurrence in the newly transplanted liver has not so far been reported.

Colectomy for concurrent ulcerative colitis does not effect the outcome of liver damage.

Note that aout 20 per cent of patients will develop a dominant biliary stricture so it is important to exclude a cholangiocarcinoma (which is common) (1).

Reference:

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