Department of Medicine Huddinge, Functional Unit Endoscopy, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden; European Reference Network for Hepatological Diseases (ENR RARE-LIVER), Sweden.
Department of Medicine and Martin Zeitz Center for Rare Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, Germany; European Reference Network for Hepatological Diseases (ENR RARE-LIVER), Sweden.
Eur J Med Genet. 2021 Jun;64(6):104231. doi: 10.1016/j.ejmg.2021.104231. Epub 2021 Apr 24.
Primary sclerosing cholangitis (PSC) is a rare, inflammatory cholestatic liver disease that causes biliary strictures which can lead to secondary complications. About 30-50% of PSC patients develop dominant strictures (DS) in the biliary tree, which are both the cause of jaundice and bacterial cholangitis as well as predilection spots for development of neoplastic development. Cancer is the most common cause of death in PSC. A central concern is to distinguish malignant from benign strictures, which eventually is done by invasive methods to obtain a brush cytology or biopsy sample, in most cases via endoscopic retrograde cholangiography-pancreatography (ERCP). Since medical therapies, like ursodesoxycholic acid or immunosuppressive drugs have no proven effect, therapeutic ERCP has become the primary management strategy to improve symptoms and in some patients may slow down disease progression. This article aims at outlining the current and emerging methods in ERCP in PSC patients.
原发性硬化性胆管炎（PSC）是一种罕见的炎症性胆汁淤积性肝病，可导致胆管狭窄，进而引发继发性并发症。约 30-50%的 PSC 患者会出现胆管树中的主导性狭窄（DS），DS 既是黄疸和细菌性胆管炎的病因，也是肿瘤发生的倾向部位。癌症是 PSC 患者死亡的最常见原因。主要关注点是区分恶性和良性狭窄，这最终需要通过侵入性方法获得刷状细胞学或活检样本来完成，在大多数情况下，该方法是通过经内镜逆行胰胆管造影术（ERCP）进行的。由于熊去氧胆酸或免疫抑制药物等医学疗法没有被证实有效，治疗性 ERCP 已成为改善症状的主要治疗策略，在某些患者中，还可能减缓疾病进展。本文旨在概述 PSC 患者 ERCP 的现有和新兴方法。
