title: The X-factor in primary biliary cirrhosis: monosomy X and xenobiotics.
abstract: Primary biliary cirrhosis (PBC) is a chronic, cholestatic, autoimmune liver disease characterised by the destruction of small- and medium-sized bile ducts. The serological hallmark of PBC includes antimitochondrial antibodies (AMA). The disease has a striking female predominance, and primarily affects women of middle-age. First-degree relatives, and in particular female relatives, are known to have an increased risk of developing the disease. Several studies have attempted to explain the female predominance of PBC, and autoimmune diseases in general. Two components that are of interest in PBC include monosomy X and xenobiotics. Monosomy X has been noted to be prevalent in the peripheral blood mononuclear cells of PBC patients. Xenobiotics, which are exogenous chemicals not normally found within the body, have been implicated in the modification of, and loss of, tolerance to AMA. Several cosmetics are known to contain these xenobiotics, which is of interest given the information provided in regards to known risk factors for PBC development. This review will focus on X monosomy and xenobiotics, which appear to constitute the X-factor of PBC.
authors.affiliation: Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Via Manzoni 56, 20089 Rozzano (MI), Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Bianchi
authors.affiliation: Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Via Manzoni 56, 20089 Rozzano (MI), Italy.
authors.forename: Ana
authors.initials: A
authors.lastname: Lleo
authors.affiliation: Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Via Manzoni 56, 20089 Rozzano (MI), Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Bernuzzi
authors.affiliation: Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Via Manzoni 56, 20089 Rozzano (MI), Italy.
authors.forename: Lisa
authors.initials: L
authors.lastname: Caliari
authors.affiliation: Institute of Liver Transplantation, Division of Transplantation Immunology and Mucosal Biology, King's College London School of Medicine at King's College Hospital, London, SE5 9RJ UK.
authors.forename: Dan S
authors.initials: DS
authors.lastname: Smyk
authors.affiliation: Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Via Manzoni 56, 20089 Rozzano (MI), Italy ; Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, Davis, USA.
authors.forename: Pietro
authors.initials: P
authors.lastname: Invernizzi
keywords: Autoimmune disease; Autoimmunity; Genetics; Monosomy X; Risk factor; Susceptibility; Xenobiotics