Treatment

Current treatment strategies focus on symptom relief, management of complications, and timely referral for liver transplantation, which remains the only definitive cure.

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.

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 about 20 per cent of patients will develop a dominant biliary stricture so it is important to exclude a cholangiocarcinoma (which is common). Patients with PSC who develop cholangiocarcinoma may also be considered for a liver transplant if they fit certain well-defined criteria. (2)

Reference:

1. European Association for the Study of the Liver. EASL Clinical Practice Guidelines on sclerosing cholangitis. J Hepatol. 2022 Sep;77(3):761-806
2. Chapman MH et al. British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut. 2019 Aug;68(8):1356-1378