Clinical Research Directory

Inclusion Criteria: * Age ≥ 18 and \< 80 years * Diagnosis of PBC based on at least 2 of the following criteria (EASL clinical practice guidelines 2017): * Elevated ALP level * Presence of antimitochondrial antibody (immunofluorescence titer ≥ 1:40 or positive antigen-specific test), specific antinuclear immunofluorescence (nuclear dots or perinuclear rims) or positive antigen-specific test for anti-gp210 or anti-Sp100 antibodies * Records of histologic features suggestive of, or compatible with PBC * UDCA therapy for the past 12 months (stable dose ≥ 12 mg/kg/d for ≥ 3 months prior to inclusion). * Non-optimal response to UDCA defined by at least one of the following criteria (ratios of absolute values to ULN rounded to the first decimal digit) observed at least 2 times at ≥ 4 weeks interval in the past 3 months, including at the inclusion visit assessment: * ALP \> 1.0 xULN * GGT \> 3.0 xULN * ALT or AST \> 1.0 xULN * Total and conjugated bilirubin \> 1.0 xULN * Women of childbearing potential must use at least one barrier contraceptive during the study and for at least 90 days after the last dose. * Affiliation to a social security system (AME excepted). * Signed informed consent. Exclusion Criteria: * Any of the following signs of advanced chronic liver disease: * Total bilirubin \> 2.0 xULN * Serum albumin \< 32 g/l * Platelet count \< 100,000/mm3 * INR \> 1.3 or prothrombin index \< 60% * Child-Pugh score B or C * MELD score ≥ 14 * History ≤ 24 months or presence of cirrhotic decompensation * Patients on the waiting list for LT * GFR estimated by CKI-EPI equation \< 60 mL/min * CPK \> 5.0 xULN * AST or ALT \> 3.0 xULN * History of LT * Autoimmune hepatitis (AIH) overlap syndrome defined by at least 2 of the following 3 criteria including the histologic one: * ALT \> 5.0 xULN * IgG \> 20 g/l or presence of anti-smooth muscle or anti-SLA antibodies * Histologic features characteristic of, or compatible with AIH * Any other chronic hepatic comorbidities (HCV, HBV, NASH, alcoholic liver disease, hemochromatosis, Wilson's disease, α1-antitrypsin deficiency, celiac disease) * Untreated hypo or hyperthyroidism (Hashimoto or Graves autoimmune thyroiditis) * Conditions that may cause non-hepatic increases in ALP (Paget's disease, osteodystrophy, hyperparathyroidism, dysglobulinemia) * Gilbert's syndrome or chronic hemolysis (hyperbilirubinemia with an unconjugated to total bilirubin ratio ≥ 75%) * History of or established or suspected hepatocellular carcinoma * History of malignancy diagnosed or treated within 2 years (recent localized treatment of squamous or non-invasive basal skin cancers is permitted) * Any severe comorbidity that may reduce life expectancy ≤ 2 years * Pregnancy or lactating * Known intolerance to bezafibrate * Known hypersensitivity to bezafibrate, any of the components of Befizal© or other fibrates * Known photosensitivity reactions or photoallergy reactions to fibrates * Patient with congenital galactosemia, glucose malabsorption, or lactase deficiency because of presence of lactose in LP tablets of bezafibrate * Participation in any other interventional study in the past 6 months * Any of the following medications used in the past 3 months before inclusion: bezafibrate, fenofibrate, ciprofibrate, gemfibrozil, obeticholic acid, budesonide, any other systemic corticosteroids, azathioprine, mycophenolate mofetil, cyclosporine, tacrolimus, sirolimus, everolimus, methotrexate. * Use of statins in the month before inclusion