原发性胆汁性胆管炎合并干燥综合征的免疫机制及靶向治疗研究进展

董笑瀛; 翟永贞

doi:10.12449/JCH250627

原发性胆汁性胆管炎合并干燥综合征的免疫机制及靶向治疗研究进展

DOI: 10.12449/JCH250627

董笑瀛,
翟永贞^, ,

中国医科大学附属盛京医院感染科，沈阳 110004

基金项目:

辽宁省自然科学基金计划项目 (2023-MS-189)

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：董笑瀛负责查阅文献，撰写论文；翟永贞负责拟定文章思路，修改并最终定稿。

详细信息

通信作者:
翟永贞， zhaiyz@sj-hospital.org （ORCID： 0000-0002-7665-9683）

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出版历程
- 收稿日期: 2024-11-11
- 录用日期: 2025-01-10
- 出版日期: 2025-06-25

Research advances in immunopathogenesis and targeted therapeutic strategies for primary biliary cholangitis comorbid with Sjögren’s syndrome

Xiaoying DONG,
Yongzhen ZHAI^{,
,}

Department of Infectious Diseases，Shengjing Hospital of China Medical University，Shenyang 110004，China

Research funding:

Natural Science Foundation Project of Liaoning Province (2023-MS-189)

More Information

Corresponding author: ZHAI Yongzhen， zhaiyz@sj-hospital.org （ORCID： 0000-0002-7665-9683）

摘要

摘要: 原发性胆汁性胆管炎（PBC）和干燥综合征（SS）均为上皮组织受累的自身免疫性疾病，且时常合并发生，可能存在共同的发病机制。目前，尚无针对PBC和SS的特异性靶向治疗方法，针对系统受累的治疗方法主要借鉴其他自身免疫性疾病的治疗方案。本文总结了PBC和SS发病机制中已经阐明的许多潜在的治疗靶点，指出PBC和SS共同的免疫病理机制中可能存在两种疾病目前和未来的靶向治疗选择，从而为开发新疗法提供有价值的思路。
- 原发性胆汁性胆管炎 /
- 干燥综合征 /
- 免疫调节 /
- 分子靶向治疗
Abstract: Primary biliary cholangitis （PBC） and Sjögren’s syndrome （SS） are both autoimmune disorders characterized by the involvement of epithelial tissue， and comorbidity of PBC and SS is often observed in clinical practice， suggesting that these two diseases may have common pathogeneses. Currently， there are still no specific targeted therapies for PBC and SS， and the therapeutic approach for systemic manifestations mainly relies on the treatment regimens for other autoimmune disorders. This article reviews the various potential therapeutic targets that have been clarified in the pathogenesis of PBC and SS and points out that targeted therapies for these two diseases can be developed based on the common immunopathological mechanism of PBC and SS， thereby providing valuable ideas for developing novel therapies.
- Primary Biliary Cholangitis /
- Sjögren’s Syndrome /
- Immunomodulation /
- Molecular Targeted Therapy

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表 1 针对PBC与SS免疫靶点的生物/化学化合物概览

Table 1. An overview of biological/chemical compounds against immunity targets in PBC and SS

药物	作用靶点	作用机制	PBC	SS
乌司奴单抗	IL-12/IL-23p40	抗IL-12/IL-23单克隆抗体	［18］	-
司库奇尤单抗	IL-17A	抗IL-17A	［23］	-
二甲双胍	AMPK	Th17和Treg动态平衡	［26］	［27］
阿巴西普	CD80/CD86	选择性T淋巴细胞共刺激调节剂	［29］	［30-34］
利妥昔单抗	CD20	抗CD20单克隆抗体	［39-40］	［42-43］
贝利尤单抗	BAFF	抗BAFF单克隆抗体	［46］	［45］
利妥昔单抗+贝利尤单抗	BAFF+CD20	抗BAFF和抗CD20联合治疗	［47］	［48］
泰它西普	BAFF+APRIL	同时抑制BAFF和APRIL	-	［50］
巴瑞替尼	JAK1/JAK2	JAK/STAT信号通路	［51］	［52-53］
非诺贝特	PPARα	TLR4/MyD88/NF-κB信号通路	［58-59］	［60］
注：“-”表示未查到相关试验文献。

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