title: Variceal bleeding in primary biliary cirrhosis patients: a subgroup with improved prognosis and a model to predict survival after first bleeding.
abstract: BACKGROUND
  Varices are a late complication in primary biliary cirrhosis (PBC). However, patients without clinical jaundice do bleed from varices; whether their prognosis differs is unknown.
  
  
  OBJECTIVE
  Evaluate PBC patients, particularly those with bilirubin <or=34 micromol/l at the time of bleeding.
  
  
  RESULTS
  One hundred and two variceal bleeders were present (median, follow-up 20.5 months, range 0-180), who at diagnosis had: pruritus (51%), fatigue (32%) and 23 (22.5%) variceal bleeding. Histologically advanced disease was present in 96 of 100 patients (stage 3: 14 and stage 4: 82); 83 died, 24 within 6 weeks of first bleeding. At the time of bleeding, 26 patients had bilirubin <or=34 micromol/l. In this group, 24 patients were stage 4, in 13 bleeding was the first presentation of PBC and they were older (59.4 vs. 55.4 years, P=0.09), had lower alkaline phosphatase (491.5 vs. 510, P=0.03) but similar albumin values, surviving a median 61 versus 12 months, compared with the 76 patients with bilirubin >34 micromol/l (P=0.001, log rank test). Hazard ratios (95% confidence intervals) for independent predictors of mortality after bleeding were: age 1.02 (1-1.05), log10 bilirubin 4.64 (2.56-8.41), ascites 2.13 (1.29-3.51) and hepatic encephalopathy 2.72 (1.56-4.74).
  
  
  CONCLUSIONS
  Variceal bleeding complicates histologically advanced PBC. A distinct subgroup with near normal bilirubin and lower alkaline phosphatase first presents with variceal bleeding in 50% of cases and has a better prognosis than jaundiced PBC variceal bleeders.
authors.affiliation: Liver Transplantation and Hepatobiliary Medicine, Royal Free Hospital, London, UK.
authors.forename: Jiannis
authors.initials: J
authors.lastname: Vlachogiannakos
authors.forename: James
authors.initials: J
authors.lastname: Carpenter
authors.forename: John
authors.initials: J
authors.lastname: Goulis
authors.forename: Christos
authors.initials: C
authors.lastname: Triantos
authors.forename: David
authors.initials: D
authors.lastname: Patch
authors.forename: Andrew K
authors.initials: AK
authors.lastname: Burroughs
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D001663:Bilirubin / Q000097:blood; D004812:Epidemiologic Methods; D004932:Esophageal and Gastric Varices / Q000209:etiology*; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000209:etiology*; D006801:Humans; D007565:Jaundice / Q000209:etiology; D008105:Liver Cirrhosis, Biliary / Q000150:complications*; D008297:Male; D008875:Middle Aged; D011379:Prognosis
chemical_list: D001663:Bilirubin