title: Geoepidemiology and changing mortality in primary biliary cholangitis.
abstract: Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a chronic cholestatic disease characterized by an autoimmune-mediated destruction of small and medium-sized intrahepatic bile ducts. Originally PBC was considered to be rare and almost invariably fatal, mainly because the diagnosis was made in patients presenting with advanced symptomatic disease (jaundice and decompensated cirrhosis). However, the development of a reproducible indirect immunofluorescence assay for antimitochondrial antibody made it possible to diagnose the disease at an earlier stage, and introduction of ursodeoxycholic acid therapy as the first-line therapy for PBC drastically changed PBC-related mortality. At present, patients with an early histological stage have survival rates similar to those of an age- and sex-matched control population. Although 30% of patients treated with ursodeoxycholic acid may exhibit incomplete responses, obeticholic acid and drugs currently in development are expected to be effective for these patients and improve outcomes. Meanwhile, more etiology and immunopathology studies using new technologies and novel animal models are needed to dissect variances of clinical course, treatment response, and outcome in each patient with PBC. Precision medicine that is individualized for each patient on the basis of the cause identified is eagerly awaited.
authors.affiliation: Department of Surgery, Oncology and Gastroenterology, University of Padova, via Giustiniani, 2, Padova, Italy.
authors.forename: Annarosa
authors.initials: A
authors.lastname: Floreani
authors.affiliation: Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Tanaka
authors.affiliation: Division of Gastroenterology and Hepatology, School of Medicine, University of California, Davis, Davis, CA, USA.
authors.forename: Christopher
authors.initials: C
authors.lastname: Bowlus
authors.affiliation: Division of Rheumatology, Allergy, and Clinical Immunology, School of Medicine, University of California, Davis, Davis, CA, USA. megershwin@ucdavis.edu.
authors.forename: Merrill Eric
authors.initials: ME
authors.lastname: Gershwin
mesh_terms: D000818:Animals; D002635:Chenodeoxycholic Acid / Q000031:analogs & derivatives / Q000627:therapeutic use; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D004195:Disease Models, Animal; D015195:Drug Design; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000453:epidemiology* / Q000401:mortality; D057285:Precision Medicine / Q000379:methods; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
chemical_list: D002756:Cholagogues and Choleretics; C464660:obeticholic acid; D002635:Chenodeoxycholic Acid; D014580:Ursodeoxycholic Acid
keywords: Cirrhosis; Etiology; Obeticholic acid; Precision medicine; Ursodeoxycholic acid