title: Predicting outcome in primary biliary cirrhosis.
abstract: Primary biliary cirrhosis (PBC) is a slowly progressive autoimmune liver disease that may ultimately result in liver failure and premature death. Predicting outcome is of key importance in clinical management and an essential requirement for patients counselling and timing of diagnostic and therapeutic interventions. The following factors are associated with progressive disease and worse outcome: young age at diagnosis, male gender, histological presence of cirrhosis, accelerated marked uctopenia in relation to the amount of fibrosis, high serum bilirubin, low serum albumin levels, high serum alkaline phosphatase levels, esophageal varices, hepatocellular carcinoma (HCC) and lack of biochemical response to ursodeoxycholic acid (UDCA). The prognostic significance of symptoms at diagnosis is uncertain. UDCA therapy and liver transplantation have a significant beneficial effect on the outcome of the disease. The Mayo risk score in PBC can be used for estimating individual prognosis. The Newcastle Varices in PBC Score may be a useful clinical tool to predict the risk for development of esophageal varices. Male gender, cirrhosis and non-response to UDCA therapy in particular, are risk factors for development of HCC.
authors.affiliation: Dept. of Gastroenterology and Hepatology, Erasmus University Medical Centre, Rotterdam, The Netherlands.
authors.forename: Willem J
authors.initials: WJ
authors.lastname: Lammers
authors.affiliation: Liver Center of Excellence, Digestive Disease Institute, Virginia Mason Medical Center, Seattle, WA, USA.
authors.forename: Kris V
authors.initials: KV
authors.lastname: Kowdley
authors.affiliation: Dept. of Gastroenterology and Hepatology, Erasmus University Medical Centre, Rotterdam, The Netherlands.
authors.forename: Henk R
authors.initials: HR
authors.lastname: van Buuren
mesh_terms: D000367:Age Factors; D000469:Alkaline Phosphatase / Q000097:blood; D001663:Bilirubin / Q000097:blood; D006528:Carcinoma, Hepatocellular / Q000209:etiology; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D003661:Decision Support Techniques; D004932:Esophageal and Gastric Varices / Q000209:etiology; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000150:complications / Q000628:therapy*; D017093:Liver Failure; D008113:Liver Neoplasms / Q000209:etiology; D016031:Liver Transplantation; D008297:Male; D011379:Prognosis; D012307:Risk Factors; D012709:Serum Albumin; D012737:Sex Factors; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
chemical_list: D002756:Cholagogues and Choleretics; D012709:Serum Albumin; D014580:Ursodeoxycholic Acid; D000469:Alkaline Phosphatase; D001663:Bilirubin