Background: Primary biliary cirrhosis is a cholestatic liver disease characterized by immune-mediated destruction of bile ducts. Its pathogenesis is largely unknown, although complex interactions between environment and genetic predisposition are proposed. Aims: Identify disease risk factors using a detailed patient questionnaire and compare study findings to 3 published reports. Methods: Questionnaire data were prospectively collected from 522 cases and 616 controls of the Mayo Clinic Primary Biliary Cirrhosis Genetic Epidemiology Registry. Case and control responses were compared using logistic regression, adjusting for recruitment age, sex, and education level. Results: Cases reported ever regularly smoking cigarettes more frequently than controls (P<. 0.001). History of urinary tract infection was similar between groups; however, cases reported multiple urinary tract infections more commonly than controls (P<. 0.001). Frequency of other autoimmune disease was higher in cases than controls (P<. 0.001). As well, prevalence of primary biliary cirrhosis among first-degree relatives was higher in case families than control families (P<. 0.001). Conclusions: Our study confirms prior reported risk factors associated with disease risk. Given the potential importance of gene and environment interactions, further examination of environmental risk factors considering genetic background may provide new insight into primary biliary cirrhosis pathogenesis.
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