Presentation and mortality of primary biliary cirrhosis in older patients.

OBJECTIVES many patients with primary biliary cirrhosis present for the first time aged over 65, but it is unclear whether the disease is different in older patients. We have examined presentation and mortality in relation to age at which primary biliary cirrhosis was first suspected clinically. DESIGN we identified 1023 patients from our regional primary biliary cirrhosis database with definite or probable primary biliary cirrhosis (689 definite); 397 (39%) presented aged > or =65. Definite primary biliary cirrhosis was defined as a positive antimitochondrial antibody titre > or =1/40, abnormal liver enzymes and compatible/diagnostic histology; probable as the presence of two of these indications. RESULTS there was no difference in presenting clinical features between the older and younger groups. Older patients were significantly less likely than younger to have had liver biopsy (50% vs 78%; P < 0.001). The 1023 patients had been followed for 8561 patient years. Follow-up was shorter (5.9+/-4 vs 9.8+/-5.5 years; P < 0.001) in the older group because of higher cumulative mortality (59% vs 33%; P < 0.001). Liver-related deaths were significantly commoner in the older group (18% vs 13%; P < 0.05). The mortality ratio for liver deaths (liver deaths per year of follow-up) was 2.4 times higher in the older group (0.031 vs 0.013). CONCLUSIONS patients with primary biliary cirrhosis who are over and under 65 have similar features on presentation. The annual risk of liver death is 2.4 times higher in those presenting over 65, reaffirming the importance of age as an independent prognostic factor in an unselected primary biliary cirrhosis population.