The pathogenesis of autoimmune diseases includes a combination of genetic factors and environmental exposures including infectious agents. Infectious triggers are commonly indicated as being involved in the induction of autoimmune disease, with Epstein-Barr virus (EBV) being implicated in several autoimmune disorders. EBV is appealing in the pathogenesis of autoimmune disease, due to its high prevalence worldwide, its persistency throughout life in the host’s B lymphocytes, and its ability to alter the host’s immune response and to inhibit apoptosis. However, the evidence in support of EBV in the pathogenesis varies among diseases. Autoimmune liver diseases (AiLDs), including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC), have a potential causative link with EBV. The data surrounding EBV and AiLD are scarce. The lack of evidence surrounding EBV in AiLD may also be reflective of the rarity of these conditions. EBV infection has also been linked to other autoimmune conditions, which are often found to be concomitant with AiLD. This paper will critically examine the literature surrounding the link between EBV infection and AiLD development. The current evidence is far from being conclusive of the theory of a link between EBV and AiLD. 1. Introduction Several viruses have been considered to be triggers of autoimmunity and overt autoimmune disease [1–6]. Amongst those, Epstein-Barr virus (EBV), which is the cause of infectious mononucleosis, is unique in a sense as it has been implicated in the induction of multiple autoimmune diseases [7, 8]. These include systemic lupus erythematosus (SLE), multiple sclerosis (MS), autoimmune thyroiditis (AT), rheumatoid arthritis (RA), inflammatory bowel diseases (IBD), insulin-dependent diabetes mellitus (IDDM), Sj？gren’s syndrome (SjS), systemic sclerosis (SSc), myasthenia gravis, and autoimmune liver diseases (AiLD) [7–20]. In fact, there are very few autoimmune diseases in which EBV has not been considered as a potential trigger of immune-mediated destruction. While in some of these diseases there is growing evidence in support of the link between EBV and autoimmunity, the link is not as strong as in others, and the pathogenic involvement of EBV is a matter of heated debate. This paper will discuss the clinical and experimental data investigating the role of EBV in the pathogenesis of AiLD. As these diseases frequently co-occur with extrahepatic autoimmune diseases, we also discuss EBV’s involvement in the pathogenesis of autoimmune manifestations seen in
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