What Is New in Primary Biliary Cirrhosis?

被引:10
作者
Bogdanos, Dimitrios P. [2 ]
Gershwin, M. Eric [1 ]
机构
[1] Univ Calif Davis, Sch Med, Div Rheumatol Allergy & Clin Immunol, Davis, CA 95616 USA
[2] Kings Coll Hosp London, Univ London Kings Coll, Sch Med, Inst Liver Studies Transplantat Immunol & Mucosal, London, England
关键词
Autoimmunity; Autoimmune disease; Bile ducts; Cholestasis; Liver; Immunology; Rheumatology; HUMAN-LEUKOCYTE ANTIGEN; RISK-FACTORS; ANTIMITOCHONDRIAL ANTIBODIES; INNATE IMMUNITY; SUSCEPTIBILITY; PATHOGENESIS; ASSOCIATION; IMMUNOPATHOGENESIS; AUTOANTIBODIES; IDENTIFICATION;
D O I
10.1159/000341118
中图分类号
R57 [消化系及腹部疾病];
学科分类号
100201 [内科学];
摘要
Primary biliary cirrhosis is a chronic cholestatic liver disease characterized by immune-mediated destruction of the small-and medium-sized intrahepatic bile ducts. The disease mainly affects middle-aged women who frequently suffer from concomitant autoimmune diseases. This review discusses the most recent findings in the epidemiology, natural history and approaches used for the accurate diagnosis of the disease. We critically review emerging data reporting on the role of genetics such as those of genome-wide association studies. Special attention is given to recent studies reporting on the immunopathogenesis of the disease and the role of plasma cells, apotopes, immunosenescence, autophagy and the involvement of components of the innate immunity in the induction of this enigmatic disease. Copyright (C) 2012 S. Karger AG, Basel
引用
收藏
页码:20 / 31
页数:12
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