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Clinical and Developmental Immunology
Volume 2012 (2012), Article ID 610504, 11 pages
http://dx.doi.org/10.1155/2012/610504
Review Article

Sex Differences Associated with Primary Biliary Cirrhosis

1Institute of Liver Studies, King’s College London School of Medicine, Denmark Hill Campus, London SE59PJ, UK
2Department of Medicine, University of Thessaly Medical School, Viopolis, 41110 Larissa, Greece
3Liver Unit, CIBEREHD, IDIBAPS, Hospital Clinic University of Barcelona, 08036 Barcelona, Spain
4Department of Microbiology and Parasitology, Faculty of Veterinary Medicine, University of Thessaly, 43100 Karditsa, Greece
5The Sheila Sherlock Liver Centre, and University Department of Surgery, Royal Free Hospital, London NW32QG, UK
6Department of Clinical Medicine, Alma Mater Studiorum, Università di Bologna, Policlinico Sant'Orsola-Malpighi, 40138 Bologna, Italy
7Center for Autoimmune Liver Diseases, Division of Internal Medicine, IRCCS Istituto Clinico Humanitas, 20089 Rozzano, Italy
8Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, Davis, CA 95616, USA
9Department of Cellular Immunotherapy and Molecular Immunodiagnostics, Institute for Biomedical Research & Technology, 41222 Larissa, Greece

Received 27 January 2012; Accepted 27 February 2012

Academic Editor: T. Nakayama

Copyright © 2012 Daniel S. Smyk et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary biliary cirrhosis (PBC) is a cholestatic liver disease of autoimmune origin, characterised by the destruction of small intrahepatic bile ducts. The disease has an unpredictable clinical course but may progress to fibrosis and cirrhosis. The diagnostic hallmark of PBC is the presence of disease-specific antimitochondrial antibodies (AMA), which are pathognomonic for the development of PBC. The disease overwhelmingly affects females, with some cases of male PBC being reported. The reasons underlying the low incidence of males with PBC are largely unknown. Epidemiological studies estimate that approximately 7–11% of PBC patients are males. There does not appear to be any histological, serological, or biochemical differences between male and female PBC, although the symptomatology may differ, with males being at higher risk of life-threatening complications such as gastrointestinal bleeding and hepatoma. Studies on X chromosome and sex hormones are of interest when studying the low preponderance of PBC in males; however, these studies are far from conclusive. This paper will critically analyze the literature surrounding PBC in males.