关键词: Cholestatic liver disease Management Obeticholic acid Primary biliary Cholangitis Primary sclerosing cholangitis Ursodeoxycholic acid

来  源:   DOI:10.14218/JCTH.2023.00519   PDF(Pubmed)

Abstract:
Cholestatic liver disease is a group of diseases in which bile acid accumulates in the liver for various reasons, resulting in abnormal liver biochemical indicators and histological damage. Cholestasis can be divided into intrahepatic cholestasis and extrahepatic cholestasis, which will contribute to liver damage and progress to liver fibrosis and cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis are the two most typical cholestatic liver diseases. Ursodeoxycholic acid is currently the first-line treatment for PBC, while obeticholic acid, budesonide and fibrates have also shown good potential in the treatment of PBC. There are currently no official drugs approved to treat primary sclerosing cholangitis, and the use of ursodeoxycholic acid may have certain clinical benefits. At present, progress has been made in new treatment directions for cholestatic liver disease, including fibroblast growth factor 19, cholestyramine, S-adenosyl-L-methionine, steroid drugs, farnesoid X receptor agonists, and more. Considerable progress has been made in the management of cholestatic liver disease but there are still many opportunities and challenges. In this review, we summarized the recommended guidelines for the management of cholestatic disease and the progress of new drug research and development, in order to provide an important reference for the clinical practice of cholestatic liver disease.
摘要:
胆汁淤积性肝病是一组胆汁酸由于各种原因在肝脏中积累的疾病,导致肝脏生化指标异常和组织学损伤。胆汁淤积可分为肝内胆汁淤积和肝外胆汁淤积,这将导致肝损伤并进展为肝纤维化和肝硬化。原发性胆汁性胆管炎(PBC)和原发性硬化性胆管炎是两种最典型的胆汁淤积性肝病。熊去氧胆酸是目前治疗PBC的一线药物,而奥贝胆酸,布地奈德和贝特类药物在治疗PBC方面也显示出良好的潜力。目前尚无官方批准用于治疗原发性硬化性胆管炎的药物,使用熊去氧胆酸可能有一定的临床益处。目前,胆汁淤积性肝病的新治疗方向取得了进展,包括成纤维细胞生长因子19,来烯胺,S-腺苷-L-蛋氨酸,类固醇药物,法尼醇X受体激动剂,还有更多.胆汁淤积性肝病的治疗取得了长足的进步,但仍存在许多机遇和挑战。在这次审查中,我们总结了胆汁淤积性疾病治疗的推荐指南和新药研发的进展,以期为胆汁淤积性肝病的临床实践提供重要参考。
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