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Primary Sclerosing Cholangitis - Treatment
Last reviewed: 04.07.2025
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There is no specific treatment for primary sclerosing cholangitis. In case of jaundice, the measures taken for chronic cholestasis and itching are recommended. Replacement therapy with fat-soluble vitamins is especially important. The advisability of systematic corticosteroid therapy has not been proven. Taking ursodeoxycholic acid improves biochemical parameters and reduces disease activity according to liver biopsy data.
Oral pulse therapy with methotrexate or colchicine is ineffective. Given the variability of the course and long asymptomatic periods, the effectiveness of treatment is difficult to evaluate clinically. Cholangitis should be treated with broad-spectrum antibiotics.
Colectomy does not affect the course of primary sclerosing cholangitis combined with nonspecific ulcerative colitis.
Endoscopic treatment allows for widening of strictures of large ducts and removal of small pigment stones or bile clots. Stents and nasobiliary catheters can be placed. Liver function tests improve, and cholangiography results are variable. Mortality is low. Controlled studies of endoscopy in primary sclerosing cholangitis have not been conducted.
Surgical treatment, such as resection of extrahepatic bile ducts and their reconstruction using transhepatic stents, is undesirable due to the high risk of developing cholangitis.
After liver transplantation in adults, the 3-year survival rate was 85%. In the bile ducts of the transplanted liver, liver strictures develop more often in patients with PSC than in patients with other diseases after transplantation.
The causes may be ischemia, rejection reaction and infection in the area of biliary anastomoses. Relapses of the disease of the transplanted liver are possible.
Cholangiocarcinomas in the transplant developed in 11 of 216 patients, the survival of these patients was very low. Given this, transplantation should be performed as early as possible.
If there is a history of operations on the bile ducts, transplantation is more difficult to perform, a large blood transfusion is required. Due to the damage to the recipient's bile duct, choledochojejunostomy is necessary. All this leads to an increase in the frequency of post-transplant complications from the bile ducts.
After transplantation, colitis often improves, but colon cancer may develop.
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