Immune-mediated liver diseases contribute significantly to morbidity and mortality due to liver failure and the need for liver transplantation. The pathogenesis of the immune-mediated chronic liver diseases, primary sclerosing cholangitis, autoimmune hepatitis, and primary biliary cirrhosis, is poorly understood. Genetic susceptibility factors may play a role, but increasing attention is being given to the association between environmental factors and these diseases. The existence of such a relationship is supported by epidemiologic surveys, animal models, and geographic clustering analyses. Unearthing the cause of this association may provide insight into the pathogenesis of immune-mediated chronic liver diseases and autoimmunity.Keywords primary sclerosing cholangitis, primary biliary cirrhosis, autoimmune hepatitis, epidemiology, geographic clustering SUMMARY Primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC), and autoimmune hepatitis (AIH) are often grouped together as immune-mediated liver diseases, though the etiology and pathogenesis of each is poorly understood. Serum antinuclear autoantibodies are common to each disease, though more frequent in AIH. Whether this loss of self-tolerance is due to cross-reactivity or self-antigen modification by an environmental toxin is unclear. The prevalence rate for each disease is low but similar (10-40/100,000 population). Overlap syndromes have been reported but account for less than 10% of cases. Despite