Practical management of primary biliary cholangitis

Primary biliary cholangitis (PBC) is a chronic, cholestatic liver disease of autoimmune pathogenesis that mainly affects middle-aged women. Patients show elevated alkaline phosphatase and bilirubin levels as the disease progresses. The main symptoms of the disease are pruritus and fatigue, which interfere with the quality of life of patients. Progressive damage leading to end-stage liver disease may require liver transplantation. Despite the efficacy of ursodeoxycholic acid (UDCA), the current standard of care for PBC, up to 40 % of patients have an inadequate response to treatment, requiring a second-line therapy. Obeticholic acid is the only second-line treatment approved for PBC in combination with UDCA in adults with inadequate response to UDCA, or as monotherapy in patients intolerant to UDCA. Although different clinical guidelines for the diagnosis and management of PBC have been published, PBC remains challenging for many physicians. In this article we briefly review the main characteristics of the disease and include a practical, user-friendly algorithm for the diagnosis and management of PBC that was developed by Spanish PBC experts based on the European Association for the Study of the Liver recommendations.