Department of Medicine IV, University of Heidelberg, Germany.
Clin Liver Dis. 2010 May;14(2):349-58. doi: 10.1016/j.cld.2010.03.010.
Primary sclerosing cholangitis is characterized by progressive fibrosing obliteration of the biliary tract. In some cases the disease is restricted to the small bile ducts, but most patients develop fibrotic stenoses of the larger bile ducts. Despite advances in magnetic resonance cholangiography, in unclear cases endoscopic retrograde cholangiography is necessary to make the correct diagnosis. In patients with total or subtotal strictures of the large bile ducts, these so-called dominant stenoses may be treated by endoscopic balloon dilatation and/or stent placement, though in the large majority of cases a stent placement is not necessary. Several studies showed an improvement of biochemical parameters after endoscopic treatment, and actuarial survival in these patients was improved compared with predicted survival. Endoscopic retrograde cholangiography allows tissue sampling, brush cytology, and bile analysis for early detection of cholangiocarcinoma, a major complication of primary sclerosing cholangitis. Despite successful endoscopic opening of bile duct stenoses, patients often progress to liver failure, leading to liver transplantation as the treatment of choice.
原发性硬化性胆管炎的特征是胆管进行性纤维性闭塞。在某些情况下，该疾病仅限于小胆管，但大多数患者会发展为较大胆管的纤维性狭窄。尽管磁共振胆管成像有所进展，但在情况不明确的情况下，仍需要进行内镜逆行胆管造影以做出正确诊断。对于大胆管完全或近完全狭窄的患者，这些所谓的主要狭窄可以通过内镜球囊扩张和/或支架置入来治疗，尽管在大多数情况下不需要支架置入。多项研究显示内镜治疗后生化参数有所改善，这些患者的生存率也得到了提高，优于预测生存率。内镜逆行胆管造影术允许进行组织取样、刷取细胞学检查和胆汁分析，以早期发现原发性硬化性胆管炎的主要并发症——胆管癌。尽管胆管狭窄的内镜治疗取得了成功，但患者常进展为肝功能衰竭，导致肝移植成为首选治疗方法。
