RESEARCH ARTICLE Open Access Primary Biliary Cirrhosis in a genetically homogeneous population: Disease associations

1Department of Gastroenterology and Hepatology, University Hospital offamilial occurrence of PBC could imply screening with AMMaria Tzardi4 and Elias A Kouroumalis1 Background: Primary biliary cirrhosis (PBC) is a disease with genetic and environmental pathogenetic background. Chemicals, infectious agents, hormone therapy, reproductive history and surgical interventions have been implicated in the induction of PBC. Familial PBC has been documented in first degree relatives (FDR). Most cohort studies are genetically heterogeneous. Our study aimed to determine eventual lifestyle or disease associations and familial occurrence rates in a genetically homogeneous and geographically defined population of PBC patients. Methods: 111 consenting PBC patients, were compared with 115 FDR and 149 controls matched for age, sex, Cretan origin and residence. All participants completed a questionnaire regarding demographics, lifestyle, medical, surgical and reproductive history. Significant variables on the univariate analysis were analyzed by multivariate analysis using a forward step-wise logistic regression model. Results: Dyslipidaemia was found in 69.4 % of patients, 60 % of FDR and 40.9 % of controls (p < 0.0001 and p = 0.003 respectively), autoimmune diseases in 36.9 % of patients, 30.4 % of FDR and 13.4 % of controls (p < 0.0001 and p = 0.011 respectively). Hashimoto’s disease (p = 0.003), Raynaud syndrome (p = 0.023) and Sjögren syndrome (p = 0.044) were significantly associated with PBC. On multivariate analysis statistically significant associations wer