原发性胆汁性胆管炎的免疫学发病机制

赵志斌; 廉哲雄

doi:10.3969/j.issn.1001-5256.2017.11.012

原发性胆汁性胆管炎的免疫学发病机制

DOI: 10.3969/j.issn.1001-5256.2017.11.012

华南理工大学医学院/生命科学研究院慢性疾病实验室

详细信息

中图分类号: R575.22
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出版历程
- 收稿日期: 2017-08-24
- 出版日期: 2017-11-20

Immunological pathogenesis of primary biliary cholangitis

Chronic Disease Laboratory, Institutes for Life Sciences and School of Medicine, South China University of Technology

摘要

摘要: 原发性胆汁性胆管炎（PBC）是一种由于免疫系统对自身肝脏进行攻击而产生的慢性胆汁淤积性自身免疫性疾病,多发于中年女性。其病理学特征表现为天然免疫细胞（NK细胞、NK/T淋巴细胞和单核细胞等）和适应性免疫细胞（T淋巴细胞和B淋巴细胞）在汇管区大量浸润,导致肝内小胆管损伤。PBC患者血清中存在高滴度的抗线粒体抗体以及较高水平的炎性细胞因子如IFNγ、TNFα、IL-6、IL-12等。目前研究表明,自身反应性T淋巴细胞是PBC致病的主要效应细胞,但是门静脉周围浸润的NK细胞、NK T淋巴细胞、单核细胞和B淋巴细胞等也直接或者间接参与了PBC的发生和发展。系统总结了天然免疫细胞、适应性免疫细胞以及相关趋化因子在PBC发病过程中的作用,为深入理解PBC的免疫学发病机制提供理论基础。
- 胆管炎,胆汁性 /
- 免疫,细胞 /
- 趋化因子
Abstract: Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune disease caused by the attack of the liver by the immune system and is commonly seen in middle-aged women.The pathological features of this disease include the infiltration of a large number of innate immune cells (NK cells, NK T cells, and monocytes, etc.) and adaptive immune cells (T lymphocytes and B lymphocytes) at the portal area and injury of small intrahepatic bile ducts.In addition, PBC patients have high serum levels of anti-mitochondrial antibodies and inflammatory cytokines such as interferon-γ, tumor necrosis factor-α, interleukin-6, and interleukin-12.Although current studies have shown that autoreactive T cells are the major effector cells for PBC, NK cells, NK T cells, monocytes, and B cells around the portal vein also directly or indirectly participate in the development and progression of PBC.This article systematically summarizes the role of innate immune cells, adaptive immune cells, and related chemokines in the pathogenesis of PBC, in order to provide a theoretical basis for a deep understanding of the immunological pathogenesis of PBC.
- cholangitis, biliary /
- immunity, cellular /
- chemokines

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