Use of Rifampin for Severe Pruritus in Children with Chronic Cholestasis

Abstract

ABSTRACTBackground:Rifampin has been proposed to reduce pruritus in children and adults with chronic cholestasis; however, there is a paucity of published data regarding the use of rifampin in children.Methods:In an open trial, 24 children were evaluated during a 6‐year period. Diagnoses included 13 patients with extrahepatic biliary atresia (54%), six with Alagille's syndrome, three with Byler's disease, and one each with primary sclerosing cholangitis and α1‐antitrypsin deficiency. All patients had severe pruritus deficiency. All patients had severe pruritus that had not responded adequately to at least 2 months of therapy with ursodeoxycholic acid, diphenhydramine, or phenobarbital and local skin care measures. Treatment was initiated with rifampin, 10 mg/kg per day in two divided doses for 18 ± 20 months, and the effect on the severity of pruritus was assessed by a clinical scoring system.Results:Ten patients showed a complete response, 12 a partial response, and 2 no response. Complete response was more common in extrahepatic cholestasis (64% vs. 10%), whereas partial response was more common in intrahepatic cholestasis (80% vs. 29%). Treatment was associated with reduction of γ‐glutamyl transpeptidase. No clinical or biochemical toxicity of rifampin was observed.Conclusions:We conclude that for more than 90% of children with chronic cholestasis and severe pruritus unresponsive to other treatments, rifampin appears to be a safe and effective therapy.