Treatment of primary sclerosing cholangitis

The natural course of primary sclerosing cholangitis (PSC) is highly variable. There is no correlation between the PSC activity and the severity of the bowel disease which occurs simultaneously in more than half of the patients. With this variability in the natural history of the disease, the clinician is faced with difficult decisions regarding the timing of medical and interventional therapy as well as of orthotopic liver transplantation. Immunosuppression is unlikely to be effective in patients with advanced liver disease with an irreversible bile duct loss. Ursodeoxycholic acid (UDCA) is now commonly prescribed in PSC since the drug is safe in most patients. High-dose UDCA treatment looked promising in a small pilot study. Therefore this approach probably in combination with immunosuppressive treatment should be investigated in prospective long term controlled trials in patients in the prefibrotic stage. Treatment of the PSC symptoms (itching, steatorrhoea, osteoporosis, bacterial cholangitis) is of paramount importance for the quality of life of the patients. Relapsing cholangitis must be treated aggressively with appropriate antibiotics e.g. ciprofloxacine or mezlocillin plus metronidazole. Balloon dilation and short term stenting will help patients with dominant stenoses of the extrahepatic bile ducts. In late-stage PSC liver transplantation is the only therapeutic option. The 5-year survival rate is good (about 80%). Orthotopic liver transplantation should be performed early in the course of the disease when the operative risk is less. At this stage-patients are also less likely to have developed hepatobiliary malignancy. The incidence of colorectal carcinoma in PSC patients with ulcerative colitis is high. Therefore, annual surveillance colonoscopy is recommended in this group of patients.