原发性硬化性胆管炎发病机制的研究进展
收稿日期: 2024-10-12
修回日期: 2025-09-02
录用日期: 2025-12-03
网络出版日期: 2025-12-17
基金资助
2025年三亚市人才开发专项资金项目(46020021T000000016220)
Research Progress on the Pathogenesis of Primary Sclerosing Cholangitis
Received date: 2024-10-12
Revised date: 2025-09-02
Accepted date: 2025-12-03
Online published: 2025-12-17
陈峙橦
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原发性硬化性胆管炎发病机制的研究进展
Primary sclerosing cholangitis (PSC) is a relatively rare biliary disease with a slow course, and characterized by extensive fibrosis of the intrahepatic and extrahepatic bile ducts, resulting in progressive thickening of the bile duct wall and narrowing of the lumen, ultimately leading to cirrhosis and liver failure, and liver transplantation is the only effective treatment method. The causes of PSC are highly complex, and the pathogenesis has not been fully elucidated. In recent years, many studies have shown that PSC is a progressive disease mainly driven by abnormal immune responses under the background of genetic susceptibility, based on bile duct epithelial cells, and influenced by intestinal microbiota disorders. Multiple factors (immunity, bile ducts, microbiota, bile acids, etc.) interact to form malignant outcomes, jointly promoting inflammation, bile duct destruction, and fibrosis processes, leading to the occurrence of PSC. Based on relevant literature,this study provides an overview and summary of the pathogenic factors PSC, such as immunology, gut microbiota, infection, bile acid metabolism, genetics, aiming to provide new directions and strategies for the treatment of PSC in clinical practice.
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