当原发性胆汁性胆管炎与其他结缔组织病共存时： 思考与挑战

蔡思燕; 魏祎; 王旭; 王立; 张奉春

doi:10.12449/JCH250504

当原发性胆汁性胆管炎与其他结缔组织病共存时：思考与挑战

DOI: 10.12449/JCH250504

中国医学科学院，北京协和医学院，北京协和医院风湿免疫科，国家皮肤与免疫疾病临床医学研究中心，疑难重症及罕见病国家重点实验室，风湿免疫学教育部重点实验室，北京 100730

基金项目:

中国医学科学院中央级公益性科研院所基本科研业务费专项资金 (2023-RW320-01);

中国医学科学院医学与健康科技创新工程项目 (2024-I2M-C&T-B-005)

利益冲突声明：本文不存在任何利益冲突。

作者贡献声明：蔡思燕负责文献查找，撰写论文；魏祎、王旭协助论文写作和修改；王立负责拟定写作思路，指导撰写文章并最后定稿；张奉春负责论文审核与指导。

详细信息

通信作者:
王立， wangli1@pumch.cn （ORCID： 0000-0003-0899-5703）

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出版历程
- 收稿日期: 2025-03-06
- 录用日期: 2025-04-15
- 出版日期: 2025-05-25

Primary biliary cholangitis comorbid with other connective tissue diseases： Thoughts and challenges

Department of Rheumatology and Clinical Immunology，Peking Union Medical College Hospital，Peking Union Medical College，Chinese Academy of Medical Sciences，National Clinical Research Center for Dermatologic and Immunologic Diseases，State Key Laboratory of Complex Severe and Rare Diseases，Key Laboratory of Rheumatology and Clinical Immunology，Ministry of Education，Beijing 100730，China

Research funding:

The Non-profit Central Research Institute Fund of Chinese Academy of Medical Sciences (2023-RW320-01);

CAMS Innovation Fund for Medical Science （CIFMS） (2024-I2M-C&T-B-005)

More Information

Corresponding author: WANG Li， wangli1@pumch.cn （ORCID： 0000-0003-0899-5703）

摘要

摘要: 原发性胆汁性胆管炎（PBC）是一种慢性、进展性自身免疫性肝病，常与结缔组织病（CTD）共存，这种共存可能改变PBC或CTD的自然病程或临床表型特征，并限制治疗用药的选择，导致临床诊疗决策棘手。由于涉及肝病学和风湿免疫学交叉学科，缺少大规模队列数据和深入的基础研究支持，临床对这些共存情况认识不足，相关证据较少，增加了临床诊疗的复杂性。本文总结了PBC与干燥综合征、系统性硬化症、系统性红斑狼疮、特发性炎性肌病等常见CTD的共病情况，分析相关免疫机制、临床表现、诊断难点、治疗策略以及预后情况。期待未来有更多的多学科协作，建立PBC与CTD共病队列，聚焦共病的基因背景、免疫机制及多组学研究，深入探讨发病机制和新型治疗靶点，并通过精准医学、人工智能技术等优化治疗策略，最大程度改善患者预后。
- 原发性胆汁性胆管炎 /
- 结缔组织疾病 /
- 诊断 /
- 治疗学
Abstract: Primary biliary cholangitis （PBC） is a chronic progressive autoimmune liver disease that is often comorbid with other connective tissue diseases （CTDs）， and such comorbidity can significantly alter the natural course or clinical phenotype of PBC or CTDs， limiting available therapeutic drugs and complicating clinical decision-making. Due to the involvement of the interdisciplinary subjects of hepatology， rheumatology， and clinical immunology and a paucity of large-scale cohort data and in-depth basic research， there is a limited understanding of such comorbidity in clinical practice， which increases the complexity of clinical diagnosis and treatment. This article summarizes the comorbidity of PBC with common CTDs such as Sjögren’‍s syndrome， systemic sclerosis， systemic lupus erythematosus， and idiopathic inflammatory myopathies， and analyzes related immune mechanisms， clinical manifestations， diagnostic challenges， treatment strategies， and prognosis. It is expected to establish PBC-CTD comorbidity cohorts through future multidisciplinary collaborations， focus on genetic background， immune mechanisms， and multi-omics approaches， elucidate pathogenesis and novel therapeutic targets， and improve the prognosis of patients by optimizing treatment strategies through precision medicine and artificial intelligence.
- Primary Biliary Cholangitis /
- Connective Tissue Diseases /
- Diagnosis /
- Therapeutics

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表 1 不同研究中PBC合并CTD的发生率

Table 1. Comparative analysis of connective tissue disease comorbidity in primary biliary cholangitis patients across reported studies

合并情况	中国			意大利^［5］（n=361）	美国^［6］（n=1 032）	欧美多中心^［1］（n=1 554）
合并情况	北京协和医院^［2］（n=322）	北京地坛医院^［3］（n=505）	北京友谊医院^［4］（n=985）	意大利^［5］（n=361）	美国^［6］（n=1 032）	欧美多中心^［1］（n=1 554）
合并CTD（%）	46.6	35.0	25.1	61.2	32.0	28.3
合并SS（%）	37.6	26.3	14.2	34.3	10.0	8.3
合并SSc（%）	2.8	‒	0.7	6.1	2.0	2.9
合并SLE（%）	3.7	0.4	0.6	2.2	3.0	1.7
合并IIM（%）	3.1	‒	0.2	‒	0.6	0.1
合并RA（%）	2.8	1.4	5.7	6.1	10.0	2.7
注：“‒”代表无相关数据。

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