The immunobiology of primary sclerosing cholangitis.
The immunobiology of primary sclerosing cholangitis.
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DOI：
10.1007/s00281-009-0154-7
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发表时间：
2009-09
影响因子：
9
通讯作者：
Bowlus, Christopher L.
中科院分区：
文献类型：
--
作者：
Aron, Jonathan H.;Bowlus, Christopher L.
原发性硬化性胆管炎（PSC）是一种慢性胆汁淤积性肝病，其组织学特征是存在肝内和/或肝外胆管同心、闭塞性纤维化，最终导致肝硬化。大约75%的PSC患者患有炎症性肠病。PSC的男性优势，缺乏一个明确的，致病性自身抗原，和先天免疫系统的潜在作用表明，它可能是由于免疫失调，而不是一个典型的自身免疫性疾病。然而，PSC与几种经典的自身免疫性疾病相关，迄今为止鉴定的与PSC最强的遗传联系是与人类白细胞抗原DRB 01 *03单倍型。PSC的确切免疫发病机制在很大程度上是未知的，但可能涉及通过门静脉递送到肝脏的细菌组分激活先天免疫系统。粘附分子和趋化因子的诱导导致肠淋巴细胞的募集。胆管损伤是由持续的炎症和炎性细胞因子的产生引起的。胆汁淤积和复发性继发性细菌性胆管炎可导致胆管狭窄进一步损害。目前，PSC尚无有效的治疗方法，制定合理的治疗策略需要更好地了解该病。
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease histologically characterized by the presence of intrahepatic and/or extrahepatic biliary duct concentric, obliterative fibrosis, eventually leading to cirrhosis. Approximately 75% of patients with PSC have inflammatory bowel disease. The male predominance of PSC, the lack of a defined, pathogenic autoantigen, and the potential role of the innate immune system suggest that it may be due to dysregulation of immunity rather than a classic autoimmune disease. However, PSC is associated with several classic autoimmune diseases, and the strongest genetic link to PSC identified to date is with the human leukocyte antigen DRB01*03 haplotype. The precise immunopathogenesis of PSC is largely unknown but likely involves activation of the innate immune system by bacterial components delivered to the liver via the portal vein. Induction of adhesion molecules and chemokines leads to the recruitment of intestinal lymphocytes. Bile duct injury results from the sustained inflammation and production of inflammatory cytokines. Biliary strictures may cause further damage as a result of bile stasis and recurrent secondary bacterial cholangitis. Currently, there is no effective therapy for PSC and developing a rational therapeutic strategy demands a better understanding of the disease.
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