Risk of primary biliary liver cirrhosis in patients with coeliac disease: Danish and Swedish cohort data

Background-Several case reports, hut only a few studies, have examined the coexistence of coeliac disease and primary biliary cirrhosis. Aim-To estimate the risk of primary biliary cirrhosis in two national cohorts of patients with coeliac disease in Denmark and Sweden. Methods-Through record linkage all Danish patients hospitalised with coeliac disease were followed for possible occurrence of primary biliary cirrhosis from 1 January 1977 until 31 December 1992. All patients hospitalised with coeliac disease in Sweden from 1987 to 1996 were also followed in a separate analysis. Results-A total of 896 patients with coeliac disease were identified in Denmark with a median follow up period of 9.1 years for a total of 8040 person-years at risk. Two cases of primary biliary cirrhosis were observed where 0.07 were expected, giving a standardised incidence ratio of 27.6 (95% confidence interval 2.9 to 133.5). A total of 7735 patients with coeliac disease were identified in Sweden with a median follow up period of 5.1 years for a total of 39 284 person-years at risk. Twenty two people with primary biliary cirrhosis were identified compared with 0.88 expected, giving a standard incidence ratio of 25.1 (95% confidence interval 15.7 to 37.9). Conclusion-Patients with coeliac disease are at increased risk of having primary biliary cirrhosis.