Primary Sclerosing Cholangitis (cont.)

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How is primary sclerosing cholangitis treated?

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The treatment of primary sclerosing cholangitis includes:

  • cholestyramine (Questran) or rifampin (Rifadin) to diminish itching
  • Antibiotics for infection, specifically cholangitis
  • Vitamin D and calcium to prevent bone loss (osteoporosis)
  • ERCP with balloon dilatation and/or stenting (a procedure in which the bile ducts are stretched open) for primary sclerosing cholangitis patients with dominant extra-hepatic duct strictures
  • Liver transplantation for patients with advanced cirrhosis

Medications

Many medications [such as ursodiol (UDCA), prednisone, methotrexate (Rheumatrex, Trexall), colchicine, 6-mercaptopurine, tacrolimus (Prograf), cyclosporine (Neoral, Gengraf)] have been studied to treat primary sclerosing cholangitis. Except in the case of prednisone for autoimmune hepatitis overlap form of primary sclerosing cholangitis, none of the other medications have shown a consistent benefit on survival or decreased need for liver transplantation.

UDCA

Ursodiol (UDCA) is a bile acid that is given orally and replaces other bile acids in the body. UDCA is believed to protect against damaging effects of other bile acids on the liver cells and also induce formation of antioxidants. UDCA is the most extensively studied medication for primary sclerosing cholangitis. At standard doses (15 mg/kg/day), it has been shown to improve fatigue, and improve blood levels of liver enzymes in patients with primary sclerosing cholangitis. UDCA is now considered if there is worsening pruritus or jaundice. However, there is still no conclusive evidence that UDCA actually prolongs life or decreases the need for liver transplantation in primary sclerosing cholangitis patients.

Treatment of dominant strictures

Dominant strictures are major narrowings in the extrahepatic bile ducts. Dominant strictures of the extrahepatic bile ducts occur in 7%-20% of primary sclerosing cholangitis patients. In selected primary sclerosing cholangitis patients with dominant strictures, ERCP and balloon dilatation (stretching) of the stricture can improve symptoms and abnormal blood levels of liver enzymes and bilirubin. Some doctors also believe that successful dilatation of dominant strictures decreases the risk of developing cholangitis. However, ERCP and dilatation of dominant strictures should be done in centers with highly experienced physicians. During ERCP, doctors often also perform brush cytology of the dominant strictures to exclude cholangiocarcinoma.

Surgery is another treatment for dominant extrahepatic strictures in primary sclerosing cholangitis patients. In carefully selected patients, surgical resection of the stricture followed by creation of a choledocho-jejunostomy (an artificial passage for bile formed by attaching the bile duct from above the stricture directly into the small intestine) can improve symptoms, delay liver transplantation, and lower the risk of cholangiocarcinoma. However, few surgeons recommend surgical resection of dominant strictures because they are concerned that scarring around the liver from such surgery may complicate future liver transplantation.

Liver transplantation

Even with modern management, most primary sclerosing cholangitis patients will die within 10 years of the time of diagnosis without liver transplantation. Transplantation is now the definitive treatment in primary sclerosing cholangitis patients with advanced cirrhosis and liver failure. One year survival following transplantation is 85%-90%, and five year survival is as high as 85%. Reasons for liver transplant in primary sclerosing cholangitis patients are similar to those in other forms of end-stage liver disease. They are:

  • Internal bleeding due to rupture of esophageal varices
  • Severe ascites that are refractory to medical treatment
  • Frequent episodes of bacterial cholangitis
  • Hepatic encephalopathy

The Mayo clinic devised a scoring model to help doctors predict the life span of primary sclerosing cholangitis patients not having a liver transplant. This model includes age, blood levels of bilirubin, albumin, AST and a history of bleeding from esophageal varices. The model suggests that when a patient's score estimates 6 month-survival is less than 80%, the patient should be considered for liver transplantation.

Medically reviewed by Venkatachala Mohan, MD; Board Certified Internal Medicine with subspecialty in Gastroenterology

REFERENCES:

"The prevalence, incidence and natural history of primary sclerosing cholangitis in an ethnically diverse population."
BMC Medicine. 2011
http://www.biomedcentral.com/1471-230X/11/83

"Primary sclerosing cholangitis: Epidemiology and pathogenesis"
uptodate.com


Medically Reviewed by a Doctor on 2/6/2014

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