原发性胆道胆管炎:发病机理及治疗综述。

IF 2.2 Q3 GASTROENTEROLOGY & HEPATOLOGY Annals of Gastroenterology Pub Date : 2025-03-01 Epub Date: 2025-02-28 DOI:10.20524/aog.2025.0953
Imen Jallouli, Michael Doulberis, Jannis Kountouras
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引用次数: 0

摘要

原发性胆管炎(PBC)是一种进行性自身免疫性肝病,以慢性炎症和小叶间胆管破坏为特征。其发病机制涉及遗传易感性、环境触发和免疫介导机制的复杂相互作用,特别是t辅助细胞活性,导致胆管损伤。一线治疗包括熊去氧胆酸(UDCA),它可以改善肝脏生物化学并减缓疾病进展,奥比胆酸(OCA)是无反应的一种选择。双联和/或三联治疗,包括UDCA、OCA和贝特类,在UDCA无反应的PBC患者中似乎更能获得治疗效果。新兴疗法,如过氧化物酶体增殖激活受体-α激动剂,生物制剂如达妥珠单抗和利妥昔单抗,以及实验性方法如干细胞治疗,在治疗PBC方面提供了有希望的进展。肝移植仍然是晚期病例的最后治疗选择。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Primary biliary cholangitis: a summary of pathogenesis and therapies.

Primary biliary cholangitis (PBC) is a progressive autoimmune liver disease characterized by chronic inflammation and destruction of interlobular bile ducts. Its pathogenesis involves a complex interplay of genetic predisposition, environmental triggers, and immune-mediated mechanisms, particularly T-helper cell activity, leading to bile duct damage. First-line therapy includes ursodeoxycholic acid (UDCA), which improves liver biochemistry and slows disease progression, with obeticholic acid (OCA) as an option for non-responders. Double and/or triple therapy, including UDCA, OCA, and fibrates, appears to be superior in achieving therapeutic benefits in UDCA-nonresponsive PBC patients. Emerging therapies, such as peroxisome proliferator-activated receptor-α agonists, biologics such as dacetuzumab and rituximab, and experimental approaches such as stem-cell therapy, offer promising advances in managing PBC. Liver transplantation remains a final treatment option for advanced cases.

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来源期刊
Annals of Gastroenterology
Annals of Gastroenterology GASTROENTEROLOGY & HEPATOLOGY-
CiteScore
4.30
自引率
0.00%
发文量
58
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