Sex Differences Associated with Primary Biliary Cirrhosis

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 disea...

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Main Authors: Daniel S. Smyk, Eirini I. Rigopoulou, Albert Pares, Charalambos Billinis, Andrew K. Burroughs, Luigi Muratori, Pietro Invernizzi, Dimitrios P. Bogdanos
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Clinical and Developmental Immunology
Online Access:http://dx.doi.org/10.1155/2012/610504
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author Daniel S. Smyk
Eirini I. Rigopoulou
Albert Pares
Charalambos Billinis
Andrew K. Burroughs
Luigi Muratori
Pietro Invernizzi
Dimitrios P. Bogdanos
author_facet Daniel S. Smyk
Eirini I. Rigopoulou
Albert Pares
Charalambos Billinis
Andrew K. Burroughs
Luigi Muratori
Pietro Invernizzi
Dimitrios P. Bogdanos
author_sort Daniel S. Smyk
collection DOAJ
description 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.
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spelling doaj-art-f7ec9425a7e041c3a0069c910ec59fbe2025-02-03T01:27:44ZengWileyClinical and Developmental Immunology1740-25221740-25302012-01-01201210.1155/2012/610504610504Sex Differences Associated with Primary Biliary CirrhosisDaniel S. Smyk0Eirini I. Rigopoulou1Albert Pares2Charalambos Billinis3Andrew K. Burroughs4Luigi Muratori5Pietro Invernizzi6Dimitrios P. Bogdanos7Institute of Liver Studies, King’s College London School of Medicine, Denmark Hill Campus, London SE59PJ, UKDepartment of Medicine, University of Thessaly Medical School, Viopolis, 41110 Larissa, GreeceLiver Unit, CIBEREHD, IDIBAPS, Hospital Clinic University of Barcelona, 08036 Barcelona, SpainDepartment of Microbiology and Parasitology, Faculty of Veterinary Medicine, University of Thessaly, 43100 Karditsa, GreeceThe Sheila Sherlock Liver Centre, and University Department of Surgery, Royal Free Hospital, London NW32QG, UKDepartment of Clinical Medicine, Alma Mater Studiorum, Università di Bologna, Policlinico Sant'Orsola-Malpighi, 40138 Bologna, ItalyCenter for Autoimmune Liver Diseases, Division of Internal Medicine, IRCCS Istituto Clinico Humanitas, 20089 Rozzano, ItalyInstitute of Liver Studies, King’s College London School of Medicine, Denmark Hill Campus, London SE59PJ, UKPrimary 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.http://dx.doi.org/10.1155/2012/610504
spellingShingle Daniel S. Smyk
Eirini I. Rigopoulou
Albert Pares
Charalambos Billinis
Andrew K. Burroughs
Luigi Muratori
Pietro Invernizzi
Dimitrios P. Bogdanos
Sex Differences Associated with Primary Biliary Cirrhosis
Clinical and Developmental Immunology
title Sex Differences Associated with Primary Biliary Cirrhosis
title_full Sex Differences Associated with Primary Biliary Cirrhosis
title_fullStr Sex Differences Associated with Primary Biliary Cirrhosis
title_full_unstemmed Sex Differences Associated with Primary Biliary Cirrhosis
title_short Sex Differences Associated with Primary Biliary Cirrhosis
title_sort sex differences associated with primary biliary cirrhosis
url http://dx.doi.org/10.1155/2012/610504
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