This is a Phase 3, randomized, double-blind, placebo-controlled, evaluation of the efficacy and safety of Bezafibrate in subjects with PSC and persistent cholestasis despite ursodeoxycholic acid therapy (UADC).~Design:~A multicentre, double-blind placebo controlled, randomised clinical trial~35 centers participants to the recruitment (French Network of Reference and Competence Centers for Rare Diseases: inflammatory biliary diseases and autoimmune hepatitis (MIVBH), including Saint-Antoine hospital, Paris as reference coordinator center)~Sample size :~104 patients, 52 in each group~Treatments groups:~UADC therapy (15-20 mg/kg/d) + Bezafibrate (400mg/d)~UDCA therapy (15-20 mg/kg/d) + placebo of bezafibrate (400mg/d)~Treatments duration :~24 months~Assessement:~Study visits at Inclusion, (M0) Randomisation and then every 3 months until M24~This is a phase III randomized, double blinded, multicenter, study.~No interim analysis is planned. Analysis will be performed at the end of the study after data review and freezing of data base according to intent to treat principle.
The objectives of this study are to evaluate the effect of bezafibrate treatment compared to placebo on efficacy and safety in patients with primary sclerosing cholangitis (PSC) and persistent cholestasis despite ursodeoxycholic acid therapy