暂无摘要。

Primary sclerosing cholangitis (PSC) is the classical hepatobiliary manifestation of inflammatory bowel disease (IBD) and a lead indication for liver transplantation (LT) in the western world. In this article, we present a Consensus Statement on LT practice, developed by a dedicated Guidelines\' Taskforce of the European Society of Organ Transplantation (ESOT). The overarching goal is to provide practical guidance on commonly debated topics, including indications and timing of LT, management of bile duct stenosis in patients on the transplant waiting list, technical aspects of transplantation, immunosuppressive strategies post-transplant, timing and extension of intestinal resection and futility criteria for re-transplantation.

What are the new contents of the guideline since 2010?A.Patients with primary and non-primary sclerosing cholangitis (PSC) are included in these guidelines for the diagnosis and management of cholangiocarcinoma.B.Define \"related stricture\" as any biliary or hepatic duct stricture accompanied by the signs or symptoms of obstructive cholestasis and/or bacterial cholangitis.C.Patients who have had an inconclusive report from MRI and cholangiopancreatography should be reexamined by high-quality MRI/cholangiopancreatography for diagnostic purposes. Endoscopic retrograde cholangiopancreatography should be avoided for the diagnosis of PSC.D. Patients with PSC and unknown inflammatory bowel disease (IBD) should undergo diagnostic colonoscopic histological sampling, with follow-up examination every five years until IBD is detected.E. PSC patients with IBD should begin colon cancer monitoring at 15 years of age.F. Individual incidence rates should be interpreted with caution when using the new clinical risk tool for PSC for risk stratification.G. All patients with PSC should be considered for clinical trials; however, if ursodeoxycholic acid (13-23 mg/kg/day) is well tolerated and after 12 months of treatment, alkaline phosphatase (γ- Glutamyltransferase in children) and/or symptoms are significantly improved, it can be considered to continue to be used.H. Endoscopic retrograde cholangiopancreatography with cholangiocytology brushing and fluorescence in situ hybridization analysis should be performed on all patients suspected of having hilar or distal cholangiocarcinoma.I.Patients with PSC and recurrent cholangitis are now included in the new unified network organ sharing policy for the end-stage liver disease model standard.J. Liver transplantation is recommended after neoadjuvant therapy for patients with unresectable hilar cholangiocarcinoma with diameter < 3 cm or combined with PSC and no intrahepatic (extrahepatic) metastases.
【更新要点】： 自2010年至今指南有哪些新内容？A.纳入原发性和非原发性硬化性胆管炎（primary sclerosing cholangitis，PSC）患者的胆管癌的诊断和管理指引。B.引入术语相关狭窄一词，定义为与梗阻性胆汁淤积和/或细菌性胆管炎的体征或症状相关的任何胆总管或肝管的胆道狭窄。C.对于磁共振成像和胰胆管造影结果不明确的患者，出于诊断目的，应复查高质量的磁共振成像/胰胆管造影。应避免为了诊断PSC进行内镜逆行胰胆管造影。D.对于未知炎症性肠病（inflammatory bowel disease，IBD）的PSC患者，应进行诊断性结肠镜组织学取样检查，每5年复查1次，直至检测到IBD。E.对于合并IBD的PSC患者，应从15岁开始进行结肠癌监测。F.PSC的临床风险新工具可用于风险分层，但应谨慎解释个体发生率。G.所有PSC患者应考虑参与临床试验；然而，如果熊去氧胆酸（13~23 mg·kg(-1)·d(-1)）耐受良好，且在治疗12个月后碱性磷酸酶(儿童中的γ-谷氨酰转移酶)和/或症状有显著改善，则可以考虑继续使用。H.所有疑似肝门部或远端胆管癌的患者均应进行内镜逆行胰胆管造影胆道细胞学刷检和荧光原位杂交分析。I.除了PSC和复发性胆管炎的患者，终末期肝病模型标准有统一的网络器官共享新政策。对于不可切除的肝门部胆管癌，直径< 3 cm或合并PSC且无肝内(外)转移的患者，建议新辅助治疗后进行肝移植。.

Management of primary or secondary sclerosing cholangitis is challenging. These Clinical Practice Guidelines have been developed to provide practical guidance on debated topics including diagnostic methods, prognostic assessment, early detection of complications, optimal care pathways and therapeutic (pharmacological, endoscopic or surgical) options both in adults and children.

In 2015, the first Chinese consensus on the diagnosis and management of primary sclerosing cholangitis was issued. In the past years, more data have emerged from the literature. Herein, the Autoimmune Liver Disease Group of the Chinese Society of Hepatology organized an expert group to review the evidence and updated the recommendations to formulate the guidelines. There are 21 recommendations on PSC clinical practice. To facilitate the differentiation between PSC and IgG4-related sclerosing cholangitis, 10 recommendations on IgG4-SC are also attached. These guidelines aim to provide a working reference for the management of PSC and IgG4-SC.
2015年我国第一个原发性硬化性胆管炎（PSC）的专家共识颁布。近年来PSC的临床研究提供了PSC新的研究数据和资料。为此，中华医学会肝病学分会自身免疫性肝病学组组织专家组对近年来的文献证据进行了评估，制定了本指南。本指南共有PSC推荐意见21条。为了利于鉴别PSC和IgG4相关硬化性胆管炎（IgG4-SC），也附有IgG4-SC的10条推荐意见。本指南的目的是为临床PSC和IgG4-SC的诊治提供参考和指导。.

Autoimmune liver disease is a group of hepatobiliary inflammatory diseases mediated by abnormal autoimmunity, mainly including autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis and overlap syndrome of any two of the above diseases at the same time. The standardization of clinical application of autoantibody detection related to autoimmune liver disease is helpful to the diagnosis and classification of diseases, as well as the prediction of prognosis, disease monitoring and pathogenesis research.
自身免疫性肝病是一组由异常自身免疫介导的肝胆炎症性疾病，主要包括自身免疫性肝炎、原发性胆汁性胆管炎、原发性硬化性胆管炎及上述任何两种疾病主要特征同时出现的重叠综合征等。自身免疫性肝病相关自身抗体检测临床应用的标准化，有助于疾病的诊断与分类、预测预后、病情监测及发病机制的研究。.

暂无摘要。

暂无摘要。

These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included medical representatives from hepatology and gastroenterology groups as well as patient representatives from PSC Support. The guidelines aim to support general physicians, gastroenterologists and surgeons in managing adults with PSC or those presenting with similar cholangiopathies which may mimic PSC, such as IgG4 sclerosing cholangitis. It also acts as a reference for patients with PSC to help them understand their own management. Quality of evidence is presented using the AGREE II format. Guidance is meant to be used as a reference rather than for rigid protocol-based care as we understand that management of patients often requires individual patient-centred considerations.

The article is published based on the results of the Russian Consensus on the diagnosis and treatment of primary sclerosing cholangitis (PSC), discussed at the 44th annual Scientific Session of the CNIIG \"Personalized Medicine in the Era of Standards\" (March 1, 2018). The aim of the review is to highlight the current issues of classification of diagnosis and treatment of patients with PSC, which causes the greatest interest of specialists. The urgency of the problem is determined by the multivariate nature of the clinical manifestations, by often asymptomatic flow, severe prognosis, complexity of diagnosis and insufficient study of PSC, the natural course of which in some cases can be considered as a function with many variables in terms of the nature and speed of progression with numerous possible clinical outcomes. In addition to progression to portal hypertension, cirrhosis and its complications, PSC can be accompanied by clinical manifestations of obstructive jaundice, bacterial cholangitis, cholangiocarcinoma and colorectal cancer. Magnetic resonance cholangiography is the main method of radial diagnostics of PSC, which allows to obtain an image of bile ducts in an un-invasive way. The use of liver biopsy is best justified when there is a suspicion of small-diameter PSC, autoimmune cross-syndrome PSC-AIG, IgG4-sclerosing cholangitis. Currently, a drug registered to treat primary sclerosing cholangitis which can significantly change the course and prognosis of the disease does not exist. There is no unified view on the effectiveness and usefulness of ursodeoxycholic acid and its dosage in PSC. Early diagnosis and determination of the phenotype of PSC is of clinical importance. It allows to determine the tactics of treatment, detection and prevention of complications.