WHAT’S NEW SINCE THE 2010 GUIDELINES?

• Inclusion of guidance for the diagnosis and management of cholangiocarcinoma (CCA) in patients with and without primary sclerosing cholangitis (PSC) (Figures 5, 8, and 9).
• Introduction of the term relevant stricture, defined as any biliary stricture of the common hepatic duct or hepatic ducts associated with signs or symptoms of obstructive cholestasis and/or bacterial cholangitis (Table 1).
• In patients with equivocal MRI with cholangiopancreatography (MRI/MRCP) findings, a repeated high-quality MRI/MRCP should be performed for diagnostic purposes. Endoscopic retrograde cholangiopancreatography (ERCP) should be avoided for the diagnosis of PSC (Figure 2).
• In patients with PSC without known inflammatory bowel disease (IBD), diagnostic colonoscopy with histological sampling should be performed and may be repeated every 5 years if IBD is not initially detected.
• Colon cancer surveillance should begin at age 15 years in patients with PSC and IBD.
• New clinical risk tools for PSC are available for risk stratification, but probabilities of events in individual patients should be interpreted with caution (Figure 4 and Table 3).
• All patients with PSC should be considered for participation in clinical trials; however, ursodeoxycholic acid (13–23 mg/kg/day) can be considered and continued if well tolerated with a meaningful improvement in alkaline phosphatase (γ-glutamyl transferase in children) and/or symptoms with 12 months of treatment.
• ERCP with biliary brushings for cytology and fluorescent in situ hybridization analysis should be obtained in all patients with suspected perihilar or distal CCA.
• There is a new United Network for Organ Sharing policy regarding standardization of Model for End-Stage Liver Disease exceptions for patients with PSC and recurrent cholangitis.
• Liver transplantation following neoadjuvant therapy is recommended for patients with perihilar CCA < 3 cm in radial diameter that is unresectable or arising in the setting of PSC and in the absence of intrahepatic or extrahepatic metastasis (Figure 9).

TABLE 1. Definitions in PSC

PSC	Chronic, cholestatic liver disease likely of autoimmune origin characterized by inflammation and fibrosis of intrahepatic and/or extrahepatic bile ducts, leading to the formation of bile duct strictures, and frequently associated with IBD
Small-duct PSC	Less common variant of PSC that is characterized by typical cholestatic and histological features of PSC but with normal bile ducts on cholangiography
PSC–AIH overlap	Concurrent diagnostic features of PSC and clinical, biochemical, and histological features of AIH
Secondary sclerosing cholangitis	Biliary strictures due to identifiable causes that can result in secondary biliary cirrhosis
IgG4 sclerosing cholangitis	Biliary strictures due to elevated IgG4-positive plasma cells in tissue and serum IgG4 elevation frequently associated with pancreatic involvement
Dominant stricture	A biliary stricture on ERCP with a diameter of ≤1.5 mm in the common bile duct or of ≤1 mm in the hepatic duct
High-grade stricture	A biliary stricture on MRI with cholangiopancreatography with >75% reduction in the common bile duct or hepatic ducts
Relevant stricture	Any biliary stricture of the common bile duct or hepatic ducts associated with signs or symptoms of obstructive cholestasis and/or bacterial cholangitis

中文翻译：

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AASLD 原发性硬化性胆管炎和胆管癌实践指南

自 2010 年指南以来有何新变化？

• 包括对患有和不患有原发性硬化性胆管炎 (PSC) 的患者的胆管癌 (CCA) 的诊断和管理指南（图 5、8 和 9）。
• 术语相关狭窄的引入，定义为与阻塞性胆汁淤积和/或细菌性胆管炎的体征或症状相关的肝总管或肝管的任何胆管狭窄（表 1）。
• 对于 MRI 伴胰胆管造影 (MRI/MRCP) 结果模棱两可的患者，应重复进行高质量的 MRI/MRCP 以进行诊断。内镜逆行胰胆管造影 (ERCP) 应避免用于 PSC 的诊断（图 2）。
• 对于没有已知炎症性肠病 (IBD) 的 PSC 患者，应进行诊断性结肠镜检查和组织学取样，如果最初未检测到 IBD，则可每 5 年重复一次。
• PSC 和 IBD 患者的结肠癌监测应从 15 岁开始。
• PSC 的新临床风险工具可用于风险分层，但应谨慎解释个别患者的事件概率（图 4 和表 3）。
• 所有 PSC 患者都应考虑参加临床试验；但是，如果在 12 个月的治疗中碱性磷酸酶（儿童中的 γ-谷氨酰转移酶）和/或症状有显着改善且耐受性良好，则可以考虑并继续使用熊去氧胆酸（13-23 mg/kg/天）。
• 所有疑似肺门周围或远端 CCA 的患者都应进行ERCP 胆管刷检以进行细胞学检查和荧光原位杂交分析。
• 有一个新的器官共享联合网络政策，涉及 PSC 和复发性胆管炎患者终末期肝病异常模型的标准化。
• 新辅助治疗后的肝移植推荐用于无法切除或在 PSC 情况下出现且没有肝内或肝外转移的肝门周围 CCA 径向直径 < 3 cm 的患者（图 9）。

表 1. PSC 中的定义

PSC	慢性胆汁淤积性肝病可能是自身免疫性起源，其特征是肝内和/或肝外胆管的炎症和纤维化，导致胆管狭窄的形成，并且经常与 IBD 相关
小风道 PSC	不太常见的 PSC 变体，其特征是 PSC 的典型胆汁淤积和组织学特征，但胆道造影显示胆管正常
PSC–AIH 重叠	PSC 的并发诊断特征和 AIH 的临床、生化和组织学特征
继发性硬化性胆管炎	由于可识别的原因导致的胆道狭窄可导致继发性胆汁性肝硬化
IgG4硬化性胆管炎	由于组织中 IgG4 阳性浆细胞升高和血清 IgG4 升高通常与胰腺受累有关而导致胆道狭窄
显性狭窄	ERCP 显示胆总管直径≤1.5 mm 或肝管直径≤1 mm 的胆管狭窄
高度狭窄	胰胆管造影 MRI 显示胆管狭窄，胆总管或肝管缩小 >75%
相关狭窄	与阻塞性胆汁淤积和/或细菌性胆管炎的体征或症状相关的任何胆总管或肝管胆管狭窄