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AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma
Hepatology ( IF 12.9 ) Pub Date : 2022-09-09 , DOI: 10.1002/hep.32771 Christopher L Bowlus 1 , Lionel Arrivé 2 , Annika Bergquist 3 , Mark Deneau 4 , Lisa Forman 5 , Sumera I Ilyas 6 , Keri E Lunsford 7 , Mercedes Martinez 8 , Gonzalo Sapisochin 9 , Rachna Shroff 10 , James H Tabibian 11 , David N Assis 12
TABLE 1. Definitions in PSC
中文翻译:
AASLD 原发性硬化性胆管炎和胆管癌实践指南
表 1. PSC 中的定义
更新日期:2022-09-09
Hepatology ( IF 12.9 ) Pub Date : 2022-09-09 , DOI: 10.1002/hep.32771 Christopher L Bowlus 1 , Lionel Arrivé 2 , Annika Bergquist 3 , Mark Deneau 4 , Lisa Forman 5 , Sumera I Ilyas 6 , Keri E Lunsford 7 , Mercedes Martinez 8 , Gonzalo Sapisochin 9 , Rachna Shroff 10 , James H Tabibian 11 , David N Assis 12
Affiliation
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).
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:
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)。
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 |