Department of Gastroenterology & Hepatology, Academic Medical Center, Amsterdam, The Netherlands.
Best Pract Res Clin Gastroenterol. 2011 Apr;25 Suppl 1:S15-8. doi: 10.1016/S1521-6918(11)70005-1.
Primary sclerosing cholangitis is often regarded as an autoimmune disorder and occurs frequently in relation to inflammatory bowel disease. The ongoing fibro-obliterative process of the biliary tree ensues in liver failure or cholangiocarcinoma in 12-18 years. PSC patients with concurrent IBD are at increased risk of developing colorectal carcinoma. Ursodeoxycholic acid, which is widely prescribed in PSC, is despite intensive clinical research still not proven to halt disease progression. Nor-ursodeoxycholic acid seems promising in animal models of cholestasis. Novel compounds that are involved in the immunological axis between the gut and the liver await clinical testing in PSC.
原发性硬化性胆管炎常被视为一种自身免疫性疾病，常与炎症性肠病有关。胆道的进行性纤维性闭塞过程导致 12-18 年内发生肝衰竭或胆管癌。同时患有 IBD 的 PSC 患者发生结直肠癌的风险增加。熊去氧胆酸广泛用于 PSC 的治疗，但尽管进行了大量的临床研究，仍未能证明其能阻止疾病进展。熊去氧胆酸类似物在胆汁淤积的动物模型中显示出良好的前景。涉及肠道和肝脏之间免疫轴的新型化合物有待在 PSC 中进行临床测试。
