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. AIM: Evaluate PBC patients, particularly those with bilirubin <or=34 micromol/l at the time of bleeding. PATIENTS/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). CONCLUSION: 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.