Primary Biliary Cirrhosis: Solving the Enigma

: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease, most commonly affecting female patients between 40 and 60 years of age. Patient sera present autoantibodies against mitochondrial antigens (AMA) and elevated serum IgM. Histologic studies demonstrate progressive destruction of...

Full description

Saved in:
Bibliographic Details
Published in:Annals of the New York Academy of Sciences 2005-06, Vol.1051 (1), p.185-193
Main Authors: GIORGINI, ALESSIA, SELMI, CARLO, INVERNIZZI, PIETRO, PODDA, M.URO, ZUIN, M.SSIMO, GERSHWIN, M.ERIC
Format: Article
Language:English
Subjects:
Antibodies, Antinuclear - blood
autoantibodies
autoimmune cholangitis
Autoimmunity
Chromosomes, Human, X
environmental factors
Epithelial Cells - pathology
genetic susceptibility
Haplotypes
Humans
Immunoglobulin A - physiology
immunology
Liver Cirrhosis, Biliary - etiology
Liver Cirrhosis, Biliary - genetics
Liver Cirrhosis, Biliary - immunology
Major Histocompatibility Complex
Mitochondria - immunology
T-Lymphocytes - immunology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:: Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease, most commonly affecting female patients between 40 and 60 years of age. Patient sera present autoantibodies against mitochondrial antigens (AMA) and elevated serum IgM. Histologic studies demonstrate progressive destruction of small‐ and medium‐sized intrahepatic bile ducts and, ultimately, liver cirrhosis. The precise mechanisms leading to selective destruction of such biliary epithelial cells are still unknown, although a number of immunomediated pathways have been proposed. Genetic background is critical in determining susceptibility to the disease, although no clear association with haplotypes of the major histocompatibility complex has been identified. Molecular mimicry by either infectious agents or xenobiotics has been proposed as a means of breaking tolerance in genetically predisposed individuals, thus leading to the onset of PBC. In this review, available data and current theories regarding the immunomediated pathogenesis of PBC will be described.
ISSN:0077-8923
1749-6632
DOI:10.1196/annals.1361.060