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Medical Author:

Dennis Lee, MD

Dennis Lee, MD

Dr. Lee was born in Shanghai, China, and received his college and medical training in the United States. He is fluent in English and three Chinese dialects. He graduated with chemistry departmental honors from Harvey Mudd College. He was appointed president of AOA society at UCLA School of Medicine. He underwent internal medicine residency and gastroenterology fellowship training at Cedars Sinai Medical Center.

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Medical Editor:

Jay W. Marks, MD

Jay W. Marks, MD

Jay W. Marks, MD, is a board-certified internist and gastroenterologist. He graduated from Yale University School of Medicine and trained in internal medicine and gastroenterology at UCLA/Cedars-Sinai Medical Center in Los Angeles.

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In this Article

• What is primary sclerosing cholangitis (PSC)?
• How common is primary sclerosing cholangitis?
• What causes primary sclerosing cholangitis?
• What are the symptoms of primary sclerosing cholangitis?
• What are the complications of primary sclerosing cholangitis?
• How is the diagnosis of primary sclerosing cholangitis made?
• How is primary sclerosing cholangitis treated?
• Primary Sclerosing Cholangitis At A Glance
• Patient Comments: Primary Sclerosing Cholangitis (PSC) - Symptoms
• Find a local Gastroenterologist in your town
• Primary Sclerosing Cholangitis Index

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)
  
  
• Medications such as ursodiol (UDCA) to retard the progression of primary sclerosing cholangitis
  
  
• 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 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. In higher doses (20-30 mg/kg/day) UDCA has been shown to improve blood liver enzymes, decrease bile duct inflammation, and decrease liver scarring. However, there is still no conclusive evidence that UDCA actually prolongs life or decreases the need for liver transplantation in primary sclerosing cholangitis patients. Doctors are currently awaiting the results of a multi-center, placebo controlled trial of high dose UDCA in primary sclerosing cholangitis.

Meanwhile, most doctors are treating primary sclerosing cholangitis patients with high dose UDCA (20-30 mg/kg/day) since UDCA is safe and well tolerated, and it may improve bile duct disease and retard the development of liver cirrhosis. Moreover, at least one study showed that UDCA may decrease colon cancer risk in patients with both primary sclerosing cholangitis and chronic ulcerative colitis.

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.