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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

李丽, 刘晖, 宋文艳. IgG4相关硬化性胆管炎临床特征及诊疗过程分析[J]. 临床肝胆病杂志, 2021, 37(8): 1883-1887. DOI: 10.3969/j.issn.1001-5256.2021.08.026
引用本文: 李丽, 刘晖, 宋文艳. IgG4相关硬化性胆管炎临床特征及诊疗过程分析[J]. 临床肝胆病杂志, 2021, 37(8): 1883-1887. DOI: 10.3969/j.issn.1001-5256.2021.08.026
LI L, LIU H, SONG WY. Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis[J]. J Clin Hepatol, 2021, 37(8): 1883-1887. DOI: 10.3969/j.issn.1001-5256.2021.08.026
Citation: LI L, LIU H, SONG WY. Clinical features, diagnosis, and treatment of IgG4-associated sclerosing cholangitis[J]. J Clin Hepatol, 2021, 37(8): 1883-1887. 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
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

详细信息
    通讯作者:

    宋文艳,13611096669@163.com

  • 中图分类号: R575.7

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

Funds: 

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:   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。

     

  • 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。

    图  2  不同年份的确诊方式

    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。
    下载: 导出CSV
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