Primary biliary cirrhosis.
Primary biliary cirrhosis (PBC) is an idiopathic chronic autoimmune liver disease that primarily affects women. It is believed that the aetiology for PBC is a combination between environmental triggers in genetically vulnerable persons. The diagnosis for PBC is made when two of the three criteria are fulfilled and they are: (1) biochemical evidence of cholestatic liver disease for at least 6 month's duration; (2) anti-mitochondrial antibody (AMA) positivity; and (3) histologic features of PBC on liver biopsy. Ursodeoxycholic acid (UDCA) is the only FDA-approved medical treatment for PBC and should be administered at a recommended dose of 13-15 mg/kg/day. Unfortunately despite adequate dosing of UDCA, approximately one-third of patients does not respond adequately and may require liver transplantation. Future studies are necessary to elucidate the role of environmental exposures and overall genetic impact not only in the development of PBC, but on disease progression and variable clinical response to therapy.
Nguyen
Douglas L
DL
Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, 200 First St. SW, Rochester, MN 55905, USA. nguyen.douglas@mayo.edu
Juran
Brian D
BD
Lazaridis
Konstantinos N
KN
D000818:Animals; D019468:Disease Management; D018450:Disease Progression; D004781:Environmental Exposure; D006801:Humans; D008099:Liver / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D016031:Liver Transplantation; D008285:Major Histocompatibility Complex / Q000235:genetics; D008930:Mitochondria, Liver / Q000502:physiology; D017211:Treatment Failure; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D014580:Ursodeoxycholic Acid