Primary Sclerosing Cholangitis (cont.)
How is primary sclerosing cholangitis treated?
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.
Next: Primary Sclerosing Cholangitis At A Glance »
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