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。

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.