title: Primary biliary cirrhosis in 2014.
abstract: OBJECTIVE
  Primary biliary cirrhosis (PBC) was first described in the 1950s as a clinical syndrome of progressive cholestatic liver disease resulting from chronic inflammatory destruction of the intrahepatic bile ducts. In the 1980s, the autoimmune nature of the disease was appreciated with the discovery of disease-specific loss of immune tolerance to the pyruvate dehydrogenase complex and subsequent development of antimitochondrial antibodies and autoreactive T cells. Then, in the 1990s, multiple clinical trials demonstrating the efficacy of ursodiol as a treatment for PBC were published, although it has been clear that ursodiol is not a cure and only delays progression in some patients.
  
  
  RESULTS
  The study of PBC in the 2000s has been buoyed by two basic science advances: rapid sequencing technologies that have led to genome wide association studies, and elucidation of the role of nuclear hormone receptors in the regulation of bile salt metabolism, which has led to novel therapies under study for cholestatic diseases.
  
  
  CONCLUSIONS
  Today's clinician should be able to determine which patients with PBC are likely to progress despite treatment with ursodiol and understand the putative new bile acid and immunosuppressant treatment strategies under development, as well as be aware of the recently described genetic factors at play in the development of PBC.
authors.affiliation: Division of Digestive and Liver Diseases, University of Texas Southwestern, Dallas, Texas, USA.
authors.forename: Avegail
authors.initials: A
authors.lastname: Flores
authors.forename: Marlyn J
authors.initials: MJ
authors.lastname: Mayo
mesh_terms: D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D058607:Fibric Acids / Q000627:therapeutic use; D020022:Genetic Predisposition to Disease; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy* / Q000453:epidemiology / Q000209:etiology; D012727:Sex Characteristics; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D002756:Cholagogues and Choleretics; D058607:Fibric Acids; D007166:Immunosuppressive Agents; D014580:Ursodeoxycholic Acid