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Guidelines on the diagnosis and management of primary sclerosing cholangitis (2021)
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摘要: 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。
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关键词:
- 原发性硬化性胆管炎 /
- IgG4相关性硬化性胆管炎 /
- 诊断 /
- 治疗 /
- 实践指南
Abstract: 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. -
表 1 推荐意见的证据等级和推荐强度等级
级别 详细说明 证据质量 高(A) 进一步研究不可能改变对该疗效评估结果的可信度 中(B) 进一步研究有可能影响该疗效评估结果的可信度, 且可能改变该评估结果 低或非常低(C) 进一步研究很有可能影响该疗效评估结果的可信度, 且很可能改变该评估结果 推荐强度 强(1) 明确显示干预措施利大于弊或者弊大于利 弱(2) 利弊不确定或无论质量高低的证据均显示利弊相当 
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表 2 继发性胆管炎的病因分类
病因分类 常见病因 慢性梗阻 胆道结石胆管狭窄(手术或慢性胰腺炎继发)肝移植后吻合口狭窄良恶性肿瘤 感染性疾病 寄生虫感染病毒感染(HIV、巨细胞病毒等) 药物、毒物 意外在胆管内注入酒精或福尔马林药物性损伤(氯胺酮、塞来昔布、七氟烷、阿莫西林克拉维酸、阿托伐他汀、英夫利昔单抗等) 免疫性 IgG4相关自身胰腺炎或IgG4相关硬化性胆管炎嗜酸性粒细胞性胆管炎肥大细胞性胆管疾病淀粉样变性Bechet’s病 缺血性胆管疾病 肝移植后肝动脉血栓肝移植排斥反应肝动脉内插管化疗肝动脉栓塞化疗系统性血管炎放射损伤 缺血样胆管疾病 危重症患者继发性硬化性胆管炎(创伤、烧伤、心胸手术、呼吸系统疾病、HELLP综合征、胰腺炎、急性心肌梗死、蛛网膜下腔出血、脑出血等)
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表 3 IgG4-SC诊断标准(2020版)
诊断指标 Ⅰ. 肝内/外胆管狭窄 a. ERC b. MRCP Ⅱ. 胆道壁增厚 a. EUS/IDUS b. CT/MRI/US Ⅲ. 血清学 血清IgG水平升高(＞135 mg/dL) Ⅳ. 病理学：满足(i)-(v)的不同组合 a. 观察到(i)、(ii)和(v) b. 观察到(v) c. 观察到(i)、(ii)、(v)以及(iii)和/或(iv) (i) 明显的淋巴浆细胞浸润和纤维化 (ii) IgG4+浆细胞≥10个/HPF (iii) 席纹状纤维化 (iv) 闭塞性静脉炎 (v) 未观察到肿瘤细胞 Ⅴ. 其他脏器受累 a.Ⅰ型自身免疫性胰腺炎 b. IgG4相关性泪腺炎/唾液腺炎(Mikulicz病)、IgG4相关的腹膜后纤维化、IgG4相关的肾脏病变 Ⅵ. 糖皮质激素治疗有效 诊断 Ⅰ. 确诊 胆道成像1、2型 Ⅰa/b+Ⅱa/b+Ⅲ/Ⅵ (1) Ⅴa+ 胆道成像3、4型 Ⅰa+Ⅱa+Ⅳb+Ⅲ/Ⅵ (2) Ⅴa- 胆道成像1、2、3、4型 Ⅰa+Ⅱa+Ⅲ+Ⅳa/Ⅵ (3)明确病理诊 Ⅳc Ⅱ. 拟诊 胆道成像1、2型 Ⅰa/b+Ⅱa/b (1) Ⅴa+ 胆道成像3、4型 Ⅰa+Ⅱa+Ⅳb (2) Ⅴa- 胆道成像1、2、3、4型 Ⅰa/b+Ⅱb+Ⅵ Ⅰa+Ⅱa+Ⅳa Ⅰa+Ⅱa+Ⅲ+Ⅵ Ⅰb+Ⅱa+Ⅲ+Ⅵ Ⅲ. 疑诊 胆道成像3、4型 Ⅰa/b+Ⅱa (1) Ⅴa+ 胆道成像1、2、3、4型 Ⅰb+Ⅱb+Ⅲ (2) Ⅴa- Ⅰa+Ⅱa+Ⅲ/Ⅴb/Ⅵ Ⅰb+Ⅱb+Ⅲ+Ⅵ
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表 4 IgG4-SC与PSC鉴别要点
指标 PSC IgG4-SC 临床特征 男性多发 + ++ 青年发病 ++ + 血清IgG4水平升高 -/+ ++ 合并IBD ++ - 合并AIP或其他IgG4-RD - ++ 糖皮质激素治疗有效 - +++ 组织学 肝脏胆道组织学 “洋葱皮样”胆管纤维化 + - 席纹样胆管纤维化 - + 闭塞性血管炎 - + ＞10个IgG4+浆细胞/HPF -/+ +++ 壶腹部活检组织 - ＞10个IgG4+浆细胞/HPF +++ 影像学特征 MRCP 胆管狭窄长度 +(＜5 mm) ++(＞10 mm) 狭窄近端胆管扩张 ++ + 外周胆管枯树枝样改变 ++ - 胆管假憩室样改变 ++ - IDUS 胆管狭窄处管壁非对称增厚 ++ +/- 胆管狭窄处以外管壁增厚 - ++ 胆管壁层次消失 ++ +/- 胆管内壁不规则 + - PCOS 胆管黏膜表面血管密度 + ++ 胆管黏膜表面血管迂曲扩张 - ++ 胆管黏膜表面纤维瘢痕组织 ++ -
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