Extrahepatic autoimmune diseases in patients with autoimmune hepatitis and their relatives: A Danish nationwide cohort study

Background & Aims It is widely accepted that patients with autoimmune hepatitis have an increased prevalence of extrahepatic autoimmune diseases. It is believed that the patients' relatives share this trait, but it has not been studied in a satisfactory setting. We conducted a nationwide registry-based cohort study on this issue. Methods From Danish healthcare registries 1994-2015, we included 2745 patients with autoimmune hepatitis, 17 812 of their first- and second-degree relatives and 27 450 general population controls matched with the patients for sex and age. We compared the sex- and age-specific prevalence of extrahepatic autoimmune diseases between the patients with autoimmune hepatitis, their relatives and the controls. We computed the prevalence ratio as a measure of the relative prevalence, using the controls as reference group. Results In the patients with autoimmune hepatitis, the prevalence ratio of extrahepatic autoimmune diseases ranged from 7 to 10 until age 30 years (prevalence ratio at age 20 = 9.92; 95% confidence interval 6.21-15.83), after which it gradually decreased to about 2 (prevalence ratio at age 80 = 2.37; 95% confidence interval 1.89-3.00). Neither first- nor second-degree relatives had an increased prevalence of extrahepatic autoimmune diseases (prevalence ratio for relatives at age 20 = 1.11; 95% confidence interval 0.72-1.70; prevalence ratio at age 80 = 0.96; 95% confidence interval 0.70-1.31). Conclusions Danish patients with autoimmune hepatitis were highly prone to extrahepatic autoimmune diseases, but their relatives were not.