A Pilot Study of Xifaxan to Treat Patients With PSC

Inclusion criteria:

- Diagnosis of PSC established by all of the following criteria:

- Alkaline phosphatase >1.5 times upper limit of normal for at least 6 months
duration

- GGT >1.5 times upper limit of normal in pediatric patients

- Cholangiography demonstrating intrahepatic and/or extrahepatic biliary
obstruction, beading, or narrowing consistent with PSC

- Liver histology in the past (if available for review) with features consistent
with or diagnostic of PSC

- Both genders

- Adults: Ages 18-75 years.

- Pediatric: Weight > 40 kg

- Patient's informed consent for study participation

Exclusion criteria:

- Treatment with systematic antibiotics, Azulfidine, ursodeoxycholic acid,
corticosteroids, colchicine, methotrexate, azathioprine, cyclosporine, chlorambucil,
budesonide, pentoxifylline, tacrolimus, vitamin E or prednisone in the preceding
three months

- Active drug or alcohol use

- Prior history of allergic reaction to the antibiotics which will be used in the study

- Any condition that, in the opinion of the investigator, would interfere with the
patient's ability to complete the study safely or successfully

- Evidence of decompensated liver disease such as recurrent variceal bleeding,
refractory ascites or spontaneous hepatic encephalopathy

- Anticipated need for transplantation in one year (Mayo survival model <80% one-year
survival without transplant)

- Findings highly suggestive of liver disease of other etiology such as chronic
alcoholic liver disease, chronic hepatitis B or C infection, hemochromatosis,
Wilson's disease, 1-antitrypsin deficiency, non-alcoholic steatohepatitis, primary
biliary cirrhosis or secondary sclerosing cholangitis

- Treatment with any study medications in the preceding three months

- Pregnancy or current lactation; subjects becoming pregnant during the study despite
all the precautions will be withdrawn and referred to their primary physicians