Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management.
Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management.
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DOI：
10.1053/j.gastro.2013.06.052
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发表时间：
2013-09
期刊：
影响因子：
29.4
通讯作者：
Lindor KD
中科院分区：
文献类型：
--
作者：
Eaton JE;Talwalkar JA;Lazaridis KN;Gores GJ;Lindor KD
原发性硬化性胆管炎(PSC)于19世纪50年代中期由S首先描述，是一种表现不均的复杂肝脏。PSC的特点是慢性胆汁淤积，伴随着胆管上皮的慢性炎症，导致多灶性胆管狭窄，可影响整个胆道系统。慢性炎症会导致肝实质和胆管树的纤维化，从而导致肝硬变和恶性肿瘤。PSC的病因尚不完全清楚，这在一定程度上解释了这种疾病缺乏有效的药物治疗。然而，我们已经开始更好地了解PSC的分子发病机制。对特定的临床亚型及其进展模式的认识可以改善疾病的表型和基因分类。我们回顾了我们目前对这种神秘障碍的理解，并讨论了未来研究的重要课题。
Primary sclerosing cholangitis (PSC), first described in the mid-1850’s, is a complex liver that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis, associated with chronic inflammation of the biliary epithelium, resulting in multi-focal bile duct strictures that can affect the entire biliary tree. Chronic inflammation leads to fibrosis involving the hepatic parenchyma and biliary tree, which can lead to cirrhosis and malignancy. The etiology of PSC is not fully understood which in part explains a lack of effective medical therapy for this condition. However, we have begun to better understand the molecular pathogenesis of PSC. The recognition of specific clinical subtypes and their pattern of progression could improve phenotypic and genotypic classification of the disease. We review our current understanding of this enigmatic disorder and discuss important topics for future studies.
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影响因子：
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