Aims of treatment for
primary sclerosing cholangitis are as follows: prevention of progression of
hepatobiliary disease, reduction of symptoms and consequences of
cholestasis (
pruritus,
osteoporosis), and prevention of complications (
colorectal cancer, hepatobiliary
cancer).
Ursodeoxycholic acid (UDCA) improves biliary secretion and laboratory parameters of
cholestasis, but its effects on liver histology and survival are not clear. It reduces the incidence of dysplasias and
carcinomas of the colon in patients with
colitis and possibly has a beneficial effect on the incidence of bile duct
carcinomas. At present, UDCA represents the most promising therapeutic option. Immunosuppressive treatment has not been proven to be effective; it appears to be indicated in the overlap syndrome with
autoimmune hepatitis but may be harmful in bacterial
cholangitis. Bacterial
cholangitis is common in patients with dominant
stenoses and requires
antibiotic treatment. Endoscopic treatment of dominant
stenoses improves
cholestasis and prolongs survival in comparison to predicted survival.
Pruritus represents a problem in some patients, and
cholestyramine represents the first-line treatment. If ineffective,
opioid antagonists,
rifampin, or
ondansetron may be tried. For treatment of
osteoporosis and
osteopenia,
calcium and
vitamin D supplementation are recommended, and in selected cases,
bisphosphonates may be indicated. In patients with severe
cholestasis and coagulation defects, parenteral supplementation of
vitamin K may be indicated. During treatment, all patients should be regularly screened for colonic and bile duct
carcinomas. Patients with
cirrhosis of the liver and its complications are treated accordingly, and in end-stage
disease, liver transplantation is indicated.