Primary sclerosing cholangitis: treatment
Last reviewed: 23.04.2024
All iLive content is medically reviewed or fact checked to ensure as much factual accuracy as possible.
We have strict sourcing guidelines and only link to reputable media sites, academic research institutions and, whenever possible, medically peer reviewed studies. Note that the numbers in parentheses ([1], [2], etc.) are clickable links to these studies.
If you feel that any of our content is inaccurate, out-of-date, or otherwise questionable, please select it and press Ctrl + Enter.
There is no specific treatment for primary sclerosing cholangitis. With jaundice, activities recommended for chronic cholestasis and itching are recommended. Particularly important is replacement therapy with fat-soluble vitamins. The feasibility of systematic corticosteroid therapy has not been proven. The use of ursodeoxycholic acid improves biochemical parameters and reduces the activity of the disease according to the liver biopsy.
Oral pulse therapy with methotrexate or colchicine is ineffective. Given the variability of the course and long asymptomatic periods, the effectiveness of treatment is clinically difficult to assess. Cholangitis should be treated with broad-spectrum antibiotics.
Colectomy does not affect the course of primary sclerosing cholangitis, which is associated with ulcerative colitis.
Endoscopic treatment allows you to expand the strictures of large ducts and remove small pigmented stones or bunches of bile. It is possible to install stents and nasolabial catheters. At the same time, liver function tests improve, and the results of cholangiography are variable. Mortality is low. Controlled studies on endoscopy with primary sclerosing cholangitis have not been conducted.
Operative treatment, such as resection of extrahepatic bile ducts and their reconstruction using transhepatic stents, is undesirable because of the high risk of developing cholangitis.
After liver transplantation in adults, the 3-year survival rate was 85%. In the bile ducts of transplanted liver in patients with PSC, liver strictures develop more often than in patients with other diseases after transplantation.
The reasons for this may be ischemia, rejection reaction and infection in the area of bile duct anastomoses. Relapses of the liver transplant are possible.
Cholangiocarcinomas in the transplant developed in 11 of 216 patients, the life span of these patients was very low. Given this, transplantation should be performed as early as possible.
If there is an anamnesis of operations on the biliary tract, transplantation is more difficult to perform, and a large amount of blood needs to be transfused. In connection with the lesion of the bile duct of the recipient, choledochoinostomy is necessary. All this leads to an increase in the frequency of posttransplantation complications from the biliary tract.
After transplantation, the course of colitis often improves, but colon cancer can develop.
[