A cohort of 559 patients in Sweden who satisfied predetermined criteri
a for the diagnosis of primary biliary cirrhosis was followed with res
pect to the incidence of cancer during the period of 1958 to 1988. The
mean follow-up time from the time of primary biliary cirrhosis diagno
sis was 9.0 +/- 5.4 yr. During the follow-up period, 148 patients died
and the primary cause of death was liver insufficiency. An overall ex
cess risk for cancer, standardized incidence ratio 1.6; 95% confidence
interval, 1.1 to 2.2, was found in the cohort. In contrast to previou
s reports, we found no excess risk for breast cancer (standardized inc
idence ratio, 0.9; 95% confidence interval, 0.3 to 2.1). The number of
hepatocellular cancers in the primary biliary cirrhosis cohort did no
t significantly differ from expected (standardized incidence ratio, 2.
91; 95% confidence interval, 0.4 to 10.5).