IgG4-related sclerosing cholangitis.
IgG4-related sclerosing cholangitis.
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DOI：
10.1002/cld.642
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发表时间：
2017-07-01
影响因子：
--
通讯作者：
Barnes, Eleanor
中科院分区：
文献类型：
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作者：
Culver, Emma L;Barnes, Eleanor
免疫球蛋白G4相关硬化性胆管炎(IgG4-SC)是一种多器官慢性纤维炎性疾病的胆道表现，即IgG4相关疾病。临床上可能类似于其他胆道疾病，包括原发性硬化性胆管炎和胆管癌。IgG4-SC可导致进展性纤维硬化性疾病和肝硬变。根据临床、实验室、放射学和病理特征，没有特定的检测来做出诊断。如果在疾病的早期阶段给予类固醇和其他免疫抑制治疗，可能会导致临床和放射学的改善，但治疗方案的明确证据基础是有限的。在过去的十年里，对IgG4-SC免疫发病机制的研究取得了进展，包括人类白细胞抗原II类易感分子、循环记忆B细胞和浆母细胞、辅助性T细胞2和调节细胞、趋化因子介导的转运以及先天免疫系统的作用。
Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of a multi-organ chronic fibroinflammatory condition, IgG4-related disease. Clinically it may mimic other biliary disease, including primary sclerosing cholangitis and cholangiocarcinoma. IgG4-SC may lead to progressive fibrosclerotic disease and cirrhosis. There is no specific test to make the diagnosis, which is based on clinical, laboratory, radiological and pathological features. Steroids and other immunosuppressive therapy may lead to clinical and radiological improvement when given in the early phase of disease, but the clear evidence base for treatment regimens is limited. Over the last decade, progress has been made in understanding the immunopathogenesis of IgG4-SC, including the role of HLA class II susceptibility molecules, circulating memory B cells and plasmablasts, T helper 2 and regulatory cells, chemokine-mediated trafficking and the innate immune system.