原发性胆汁性胆管炎（primary biliary cholangitis， PBC）是一种慢性自身免疫性胆汁淤积性肝病，其发病机制尚不明确。熊去氧胆酸（ursodeoxycholic acid， UDCA）是治疗本病的一线药物，但仍有部分患者对UDCA治疗应答不佳，且不完全应答患者总体预后较差，亟须安全有效的二线治疗药物。近年来越来越多的临床试验证明中医药治疗PBC疗效确切，安全性良好，其在改善患者生化指标、提高UDCA应答率、改善临床症状、抗肝纤维化、调控免疫及提高生活质量等方面均有突出优势，但目前缺乏高质量循证医学证据以及长期终点事件研究。本文主要从中医病因病机认识及中医药治疗提高PBC疗效的相关临床研究进展进行评述，以供临床研究参考与借鉴。

Primary biliary cholangitis （PBC） is a chronic autoimmune cholestatic liver disease， and its pathogenesis is not clear. Ursodeoxycholic acid （UDCA） is the first-line drug for the treatment of this disease， but there are still some patients with poor response to UDCA， and the overall prognosis of patients with incomplete response is poor， so it is urgent to use safe and effective second-line drugs. In recent years， more and more clinicaltrials have proved that traditional Chinese medicine is effective in the treatment of PBC. It has outstanding advantages in improving patients' biochemical indicators， improving UDCA response rate， improving clinical symptoms， anti-fibrosis， regulating immunity and improving the quality of life. However， there is a lack of high-quality evidence-based medicine and long-term endpoint events research. This article primarily reviewed the progress in clinical research related to the understanding of the etiology and pathogenesis of primary biliary cholangitis （PBC） from the perspective of traditional Chinese medicine， as well as how traditional Chinese medicine can improve the therapeutic effects of PBC， providing a reference for clinical research and practice.