PSCA Pilot Study of Xifaxan in Patients With Primary Sclerosing Cholangitis

Date de début de l'étude : 1 août 2012

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months Pregnancy or current lactation; subjects becoming pregnant during the study despite all the precautions will be withdrawn and referred to their primary physicians