IgG4相关硬化性胆管炎临床特征及诊疗过程分析

李丽; 刘晖; 宋文艳

doi:10.3969/j.issn.1001-5256.2021.08.026

IgG4相关硬化性胆管炎临床特征及诊疗过程分析

DOI: 10.3969/j.issn.1001-5256.2021.08.026

李丽^a,
刘晖^b,
宋文艳^c, ,

a.
首都医科大学附属北京佑安医院中西医结合治疗中心, 北京 100069
b.
首都医科大学附属北京佑安医院病理科, 北京 100069
c.
首都医科大学附属北京佑安医院放射科，北京 100069

基金项目:

艾滋病和病毒性肝炎等重大传染病防治 (2018ZX10725504);

"扬帆计划"重点医学专业项目 (ZYLX201819)

利益冲突声明：本研究不存在研究者、伦理委员会成员、受试者监护人以及与公开研究成果有关的利益冲突。

作者贡献声明：李丽、宋文艳负责课题设计，资料分析，撰写论文；李丽、刘晖、宋文艳参与收集数据，修改论文；李丽、宋文艳负责拟定写作思路，指导撰写文章并最后定稿。

详细信息

通信作者:
宋文艳，13611096669@163.com

中图分类号: R575.7
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出版历程
- 收稿日期: 2020-12-27
- 录用日期: 2021-01-20
- 出版日期: 2021-08-20

Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis

Li LI^a,
Hui LIU^b,
Wenyan SONG^{c
, ,}

a.
Integrated Traditional Chinese and Western Medicine Treatment Center, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China
b.
Department of Pathology, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China
c.
Department of Radiology, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China

Research funding:

Major Scientific and Technological Projects for Prevention and Treatment of Major Infectious Diseases Such as AIDS and Viral Hepatitis (2018ZX10725504);

Key Medical Professional Projects of "Sailing Plan" (ZYLX201819)

摘要

摘要: 目的观察IgG4相关硬化性胆管炎临床特点, 并对其诊疗过程进行分析。方法回顾性分析2014年1月—2020年9月在首都医科大学附属北京佑安医院诊断为IgG4-SC的25例患者的临床资料，通过电子病例系统采集患者基本信息、病史、诊疗经过、实验室检查、影像、病理资料。分析临床特征、确诊流程、误诊原因等。符合正态分布的计量资料用x ±s表示，非正态分布的计量资料用M(P_最小值~P_最大值)表示。结果 25例IgG4-SC患者以男性为多见(21例，84%)，平均年龄(57.61±9.73)岁，首发症状常见皮肤黄染和/或尿黄(18例，78.26%)、乏力(9例，39.13%)等。影像学分类：Ⅰ型16例(64%)；Ⅱa型0例，Ⅱb型4例(16%)；Ⅲ型1例(4%)；Ⅳ型4例(16%)，合并胰腺受累最多见(22例，88%)。治疗主要以糖皮质激素为主，2周内TBil平均下降67.48%。确诊的22例患者中，门诊拟诊“胆胰系统占位” 15例(68.18%)，拟诊“梗阻性黄疸”5例(22.73%)。早期认识IgG4-SC疾病是通过术后病理，随后影像医师逐渐认识，最后通过多学科会诊方式使更多临床医生认识该病，患者得以更早确诊。结论对梗阻性黄疸的患者，临床医生需要结合临床表现尤其是影像学特点，对IgG4-SC进行鉴别。影像科、病理科、多学科会诊方式在帮助临床医师认识疾病、确定诊断方面发挥重要作用。
- 免疫球蛋白G /
- 胆管炎, 硬化性 /
- 诊断, 鉴别 /
- 治疗学
Abstract: Objective To investigate the clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis (IgG4-SC). Methods A retrospective analysis was performed for the clinical data of 25 patients who were diagnosed with IgG4-SC in Beijing YouAn Hospital, Capital Medical University, from January 2014 to September 2020, and an electronic medical record system was used to collect general information, medical history, diagnosis and treatment processes, laboratory examination, imaging data, and pathological data. Clinical features, process of confirmed diagnosis, and cause of misdiagnosis were analyzed. Normally distributed continuous data were expressed as mean±SD, and the non-normally distributed continuous data were expressed as M(P_{minimum value}-P_{maximum value}). Results Among the 25 patients with IgG4-SC, there were 21 (84%) male patients, and the mean age was 57.61±9.73 years. Common initial symptoms included jaundice and/or yellow urine in 18 patients (78.26%) and weakness in 9 patients (39.13%). As for imaging classification, 16 patients (64%) had type Ⅰ IgG4-SC, no patient had type Ⅱa IgG4-SC, 4 (16%) had IIb IgG4-SC, 1 (4%) had type Ⅲ IgG4-SC, and 4 (16%) had type Ⅳ IgG4-SC. Pancreatic involvement was observed in 22 patients (88%). Glucocorticoids were the main treatment method, and total bilirubin achieved a mean reduction of 67.48% within 2 weeks. Among the 22 patients with a confirmed diagnosis in our hospital, 15 (68.18%) were suspected of "space-occupying lesions in the pancreatic and biliary system" and 5 (22.73%) were suspected of obstructive jaundice at the outpatient service. Understanding of IgG4-SC was achieved by postoperative pathology in the early stage, then imaging doctors gradually became aware of this disease, and finally multidisciplinary consultation made more physicians understand the disease, which helped to make a confirmed diagnosis earlier. Conclusion For patients with obstructive jaundice, clinical physicians need to identify IgG4-SC based on clinical manifestations and especially imaging features. Multidisciplinary consultation involving imaging and pathology plays an important role in helping clinicians understand this disease and making a confirmed diagnosis.
- Immunoglobulin G /
- Cholangitis, Sclerosing /
- Diagnosis, Differential /
- Therapeutics

HTML全文

图 1 各型IgG4-SC影像学特点

注：a, Ⅰ型, 胆总管远端狭窄；b, Ⅱb型, 狭窄位于肝内胆管及胆总管远端；c, Ⅲ型, 狭窄位于肝门部及胆总管远端；d, Ⅳ型, 狭窄位于肝门部。

下载: 全尺寸图片幻灯片

图 2 不同年份的确诊方式

下载: 全尺寸图片幻灯片

表 1 25例患者基线实验室指标

基线指标	数值	正常值范围
ALT(U/L)	186.72±119.20	9~50
AST(U/L)	115.00±61.03	15~40
TBil(μmol/L)	121.63±92.91	5~21
Alb(g/L)	36.74±5.36	40~55
GGT(U/L)	605.56±498.94	10~60
ALP(U/L)	372.39±153.53	45~125
GLU(mmol/L)	7.80±2.78	3.9~6.2
IgG4(g/L)	18.45±2.95	0.03~2.01
CEA(ng/ml)	4.40±2.95	≤1.51
CA-199(U/ml)	74.49(5.00~1000.00)	0~40
CA-125(U/ml)	15.40(5.84~21.75)	≤35
注：GLU，血糖；CEA，癌胚抗原。

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