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原发性硬化性胆管炎合并炎症性肠病的治疗

Treatment of primary sclerosing cholangitis combined with inflammatory bowel disease.

作者信息

Kim You Sun, Hurley Edward H, Park Yoojeong, Ko Sungjin

机构信息

Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.

Department of Internal Medicine, Kangdong Sacred Heart Hospital, Hallym University College of Medicine, Seoul, Korea.

出版信息

Intest Res. 2023 Oct;21(4):420-432. doi: 10.5217/ir.2023.00039. Epub 2023 Sep 1.

Abstract

Primary sclerosing cholangitis (PSC) is a progressive cholestatic, inflammatory, and fibrotic disease that is strongly associated with inflammatory bowel disease (IBD). PSC-IBD represents a unique disease entity and patients with this disease have an increased risk of malignancy development, such as colorectal cancer and cholangiocarcinoma. The pathogenesis of PSC-IBD involves genetic and environmental factors such as gut dysbiosis and bile acids alteration. However, despite the advancement of disease characteristics, no effective medical therapy has proven to have a significant impact on the prognosis of PSC. The treatment options for patients with PSC-IBD do not differ from those for patients with PSC alone. Potential candidate drugs have been developed based on the pathogenesis of PSC-IBD, such as those that target modulation of bile acids, inflammation, fibrosis, and gut dysbiosis. In this review, we summarize the current medical treatments for PSC-IBD and the status of new emerging therapeutic agents.

摘要

原发性硬化性胆管炎(PSC)是一种进行性胆汁淤积性、炎症性和纤维化疾病,与炎症性肠病(IBD)密切相关。PSC-IBD代表一种独特的疾病实体,患有这种疾病的患者发生恶性肿瘤(如结直肠癌和胆管癌)的风险增加。PSC-IBD的发病机制涉及遗传和环境因素,如肠道菌群失调和胆汁酸改变。然而,尽管对疾病特征的认识有所进展,但尚无有效的药物治疗被证明对PSC的预后有显著影响。PSC-IBD患者的治疗选择与单纯PSC患者的治疗选择并无不同。基于PSC-IBD的发病机制已开发出潜在的候选药物,如那些靶向调节胆汁酸、炎症、纤维化和肠道菌群失调的药物。在本综述中,我们总结了PSC-IBD的当前药物治疗方法以及新兴治疗药物的现状。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/db62/10626010/b3ba623610ee/ir-2023-00039f1.jpg

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