内科理论与实践››2022,Vol. 17››Issue (01): 24-28.doi:10.16138/j.1673-6087.2022.01.005
杨松, 李玥
收稿日期:2022-01-21出版日期:2022-02-28发布日期:2022-07-25基金资助:
Received:2022-01-21Online:2022-02-28Published:2022-07-25
图1
日本IgG4-SC根据胆管影像分型示意图[22]
表1
IgG4-SC诊断标准（2020日本标准）[22]
| 诊断指标和标准 |
|---|
| 诊断指标 Ⅰ. 存在肝内和（或）肝外胆管狭窄证据 |
| a. ERCP提示肝内和（或）肝外胆管狭窄 |
| b. MRCP提示肝内和（或）肝外胆管狭窄 |
| Ⅱ. 存在胆管壁增厚证据 |
| a. ESU/IDUS发现胆管壁增厚证据 |
| b. CT/MRI/超声发现胆管壁增厚证据 |
| Ⅲ. 血清学检测 |
| 血清IgG4水平升高≥1 350 mg/L |
| Ⅳ. 病理证据 |
| a. 病理发现（i）+（ii）+（v） |
| b. 病理发现（v） |
| c. 在上述（i）+（ii）+（v）基础上发现（iii）和（或）（iv） |
| （i）明显的淋巴细胞浆细胞浸润和纤维化 |
| （ii）每高倍视野IgG4阳性浆细胞>10个 |
| （iii）席纹状纤维化 |
| （iv）闭塞性静脉炎 |
| （v）未见肿瘤细胞 |
| Ⅴ. IgG4相关疾病中其他脏器受累证据 |
| a. Ⅰ型AIP b. IgG4相关泪腺炎/唾液腺炎（Mikulicz病）、IgG4相关腹膜 后纤维化、IgG4相关肾脏病变 |
| Ⅵ. 糖皮质激素治疗有效 一般建议激素治疗前应完善检查排除肿瘤性疾病,建议在 激素治疗后2周完善ERCP或MRCP评价病变缓解情况, 如胆管病变无缓解要再次排除肿瘤性病变 |
| 诊断标准 |
| 以下情况可以确诊IgG4-SC |
| 满足Ⅰa/b+Ⅱa/b+Ⅲ/Ⅵ+Ⅴa可确诊1或2型IgG4-SC 满足Ⅰa+Ⅱa+Ⅳb+Ⅲ/Ⅵ+Ⅴa可确诊3或4型IgG4-SC 满足Ⅰa+Ⅱa+Ⅲ+Ⅳa/Ⅵ可确诊1~4型IgG4-SC 另外,只要组织病理符合Ⅳc也可以确诊IgG4-SC |
| 以下情况考虑IgG4-SC可能性大 |
| 满足Ⅰa/b+Ⅱa/b+Ⅴa考虑1或2型IgG4-SC可能性大 满足Ⅰa+Ⅱa+Ⅳb+Ⅴa或Ⅰa/b+Ⅱb+Ⅵ+Ⅴa考虑3或4型 IgG4-SC可能性大 满足Ⅰa+Ⅱa+Ⅳa或Ⅰa+Ⅱa+Ⅲ+Ⅳb或Ⅰb+Ⅱa+Ⅲ+Ⅵ考 虑1~4型IgG4-SC可能性大 |
| 以下情况考虑IgG4-SC不能排除 满足Ⅰa/b+Ⅱa+Ⅴa或Ⅰb+Ⅱb+Ⅲ+Ⅴa不能排除3或4型 IgG4-SC 满足Ⅰa+Ⅱa+Ⅲ/Ⅴb/Ⅵ或Ⅰb+Ⅱb+Ⅲ+Ⅵ不能排除1~4型 IgG4-SC |
表2
SSC的病因分类
| 病因分类 | 常见病因 |
|---|---|
| 慢性胆 道梗阻 |
胆总管或肝内胆管结石 手术后胆管损伤（如胆囊切除术后胆管狭窄） 肝移植术后吻合口胆管狭窄 慢性胰腺炎 良、恶性肿瘤压迫胆管 门静脉高压性胆道病等 |
| 感染性 疾病 |
复发性化脓性胆管炎（细菌） 寄生虫（隐孢子虫、微孢子虫、蛔虫、华支睾吸虫） 巨细胞病毒感染 获得性免疫缺陷综合征相关性胆管病变 感染性休克等 |
| 免疫性 疾病 |
IgG4-SC或IgG4相关AIP 嗜酸性粒细胞性胆管炎 肥大细胞性胆管病 滤泡性胆管炎 伴粒细胞上皮损害的硬化性胆管炎 淀粉样变性 骨髓移植后移植物抗宿主病等 |
| 肿瘤或炎 症等浸润 胆道系统 |
恶性肿瘤（如胆管癌等） 结节病 朗格汉斯细胞组织细胞增生症（组织细胞增生症X） |
| 缺血性胆 管疾病 |
肝动脉血栓形成（如肝移植术后） 肝移植排斥反应 放射性损伤 肝动脉化疗/栓塞 系统性血管炎 SSC-CIP |
| 药物、毒 物所致 |
手术暴露（甲醛、乙醇或高渗氯化钠溶液意外注入胆管） 药物性损伤（如氯胺酮、塞来昔布、七氟烷、阿莫西林克拉维酸、阿托伐他汀、英夫利西单抗、PD-1抑制剂等） |
| 遗传性 疾病 |
胆汁转运蛋白缺陷（如MDR3基因缺乏） 胆管板畸形/纤维多囊性肝脏疾病 囊性纤维化 CD40配体缺陷（X连锁高IgM综合征） |
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