title: Primary biliary cirrhosis in adults.
abstract: Primary biliary cirrhosis (PBC) is a chronic, autoimmune, cholestatic liver disease. It is characterized by slow destruction of small intrahepatic bile ducts, impaired biliary secretion and stasis of toxic endogenous bile acids within the liver with progression to liver fibrosis and cirrhosis. It has an increasing prevalence worldwide. It occurs more commonly in women than men at a ratio of 10:1. In most cases, diagnosis relies on a positive antimitochondrial antibody in the context of chronic cholestasis, without the need for a liver biopsy. Ursodeoxycholic acid improves survival even in patients with advanced liver disease. Certain findings such as fatigue, anti-nuclear antibodies, anti-centromere antibodies and the GP210 antinuclear antibody predict a poor outcome. Up to 40% of patients do not respond satisfactorily to ursodeoxycholic acid therapy and should be considered for adjunctive therapies. Several adjunctive and newer therapies are being tested and some appear promising. We provide a review of PBC with a focus on advances in therapies that may impact the management of PBC in the near future.
authors.affiliation: University of Kentucky, Lexington, KY, USA.
authors.forename: Njideka
authors.initials: N
authors.lastname: Momah
authors.forename: Keith D
authors.initials: KD
authors.lastname: Lindor
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000276:immunology; D002635:Chenodeoxycholic Acid / Q000031:analogs & derivatives* / Q000627:therapeutic use; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy* / Q000276:immunology; D018160:Receptors, Cytoplasmic and Nuclear / Q000819:agonists*; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*; D000099150:Receptor, Farnesoid X-Activated
chemical_list: D002635:Chenodeoxycholic Acid; D002756:Cholagogues and Choleretics; D018160:Receptors, Cytoplasmic and Nuclear; D014580:Ursodeoxycholic Acid; D000099150:Receptor, Farnesoid X-Activated; C464660:obeticholic acid