Open AccessPractical management of primary biliary cholangitis
Author(s) -
Albert Parés
Publication year - 2021
Publication title -
revista española de enfermedades digestivas/revista española de enfermedades digestivas
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.331
H-Index - 38
eISSN - 2340-4167
pISSN - 1130-0108
DOI - 10.17235/reed.2021.8219/2021
Subject(s) - obeticholic acid , medicine , ursodeoxycholic acid , liver transplantation , primary biliary cirrhosis , chronic liver disease , disease , liver disease , quality of life (healthcare) , gastroenterology , primary sclerosing cholangitis , biliary disease , intensive care medicine , transplantation , cirrhosis , receptor , nursing , agonist
Primary biliary cholangitis (PBC) is a chronic and 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 could 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 the treatment, requiring a second-line therapy. Obeticholic acid is the only second-line treatment approved for PBC in combination with UDCA in adults with an 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 is still 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 developed by Spanish PBC experts and based on the European Association for the Study of the Liver recommendations.