Department of Internal Medicine IV, University of Heidelberg, Heidelberg, Germany.
Dig Dis. 2011;29 Suppl 1:41-5. doi: 10.1159/000331074. Epub 2011 Nov 15.
Progressive destruction of bile ducts in primary sclerosing cholangitis (PSC) may lead to end-stage liver disease, portal hypertension and liver failure. The diagnosis of PSC is made by characteristic multifocal stricturing and dilation of intrahepatic and/or extrahepatic bile ducts observed by cholangiography. Magnetic resonance cholangiography is considered to have comparable accuracy to endoscopic retrograde cholangiography (ERC) in the diagnosis of PSC, but its accuracy is limited in early stages of PSC. Up to 60% of patients with PSC develop a dominant stricture of the intra- or extrahepatic biliary tree. Patients may present with jaundice, pruritus or ascending cholangitis. Therefore, in patients with an increase in serum bilirubin and/or worsening pruritus, progressive bile duct dilation on imaging studies and/or cholangitis seen via ERC, it is recommended to exclude a dominant stricture. However, in a considerable number of patients without symptoms, a dominant stricture can be detected on the cholangiogram. The cholangiographic findings and the clinical presentation make it difficult to distinguish PSC from cholangiocarcinoma. The accuracy in the distinction between these two conditions is still rather disappointing, despite the combined use of imaging, endoscopic biopsy, and cytology. Medical, endoscopic, and surgical therapies aim to slow the progression of the disease. It is generally agreed upon that patients with symptoms from dominant strictures like cholangitis, jaundice, pruritus or worsening biochemical indices are candidates for endoscopic therapy. Although the best therapeutic endoscopic approach for these patients is still under discussion, a number of reports have documented clinical and radiographic improvement in patients following endoscopic dilation with or without placement of a biliary stent. Furthermore, indirect evidence by retrospective studies suggests that endoscopic therapy may improve survival.
原发性硬化性胆管炎（PSC）中胆管的进行性破坏可能导致终末期肝病、门静脉高压和肝功能衰竭。PSC 的诊断通过胆管造影观察到的肝内和/或肝外胆管的特征性多发性狭窄和扩张来确定。磁共振胆管成像（MRCP）被认为在诊断 PSC 方面与内镜逆行胰胆管造影（ERCP）具有相当的准确性，但在 PSC 的早期阶段其准确性有限。多达 60%的 PSC 患者会出现肝内或肝外胆管树的主导性狭窄。患者可能出现黄疸、瘙痒或上行性胆管炎。因此，对于血清胆红素升高和/或瘙痒加重、影像学检查显示进行性胆管扩张和/或通过 ERCP 看到胆管炎的 PSC 患者，建议排除主导性狭窄。然而，在相当数量无症状的患者中，可以在胆管造影上检测到主导性狭窄。胆管造影表现和临床表现使得难以将 PSC 与胆管癌区分开来。尽管联合使用影像学、内镜活检和细胞学检查，但在这两种疾病的鉴别准确性仍然相当令人失望。医疗、内镜和手术治疗旨在减缓疾病的进展。普遍认为，有胆管炎、黄疸、瘙痒或生化指标恶化等主导性狭窄症状的患者是内镜治疗的候选者。尽管这些患者的最佳治疗性内镜方法仍在讨论中，但许多报告记录了内镜扩张联合或不联合胆道支架置入后患者的临床和影像学改善。此外，回顾性研究的间接证据表明，内镜治疗可能改善生存。
