中文English
ISSN 1001-5256 (Print)
ISSN 2097-3497 (Online)
CN 22-1108/R

留言板

尊敬的读者、作者、审稿人, 关于本刊的投稿、审稿、编辑和出版的任何问题, 您可以本页添加留言。我们将尽快给您答复。谢谢您的支持!

姓名
邮箱
手机号码
标题
留言内容
验证码

12例IgG4相关硬化性胆管炎临床特征分析

王艳 潘静 霍娜 张真真 程浩 王贵强

引用本文:
Citation:

12例IgG4相关硬化性胆管炎临床特征分析

DOI: 10.3969/j.issn.1001-5256.2018.06.024
详细信息
  • 中图分类号: R575.7

Clinical features of IgG4-associated sclerosing cholangitis: An analysis of 12 cases

  • 摘要:

    目的探讨分析IgG4相关硬化性胆管炎(IgG4-SC)的临床特征以及激素应答与复发情况,帮助缩短临床诊断时间并降低复发率。方法收集2011年12月-2017年3月北京大学第一医院收治的IgG4-SC患者12例,均符合2012年IgG4-SC临床诊断标准。回顾性分析临床资料、临床特点以及随访资料。结果 12例确诊患者,男女比例11∶1。平均发病年龄为(61.7±8.6)岁,平均确诊时间24.2个月。其中11例(92%)患者出现黄疸,血清TBil平均水平为(198.7±57.7)μmol/L。血清ALT水平均有不同程度升高(55453 U/L);5例(41.7%)患者急性腹痛,CA19-9平均水平(60.8±12.5)U/ml。血清IgG4升高1.518.9倍,平均(7.7±2.0)g/L。12例患者磁共振胰胆管造影均显示典型的胆管壁增厚、管腔狭窄,肝内外胆管扩张等表现,合并自身免疫性胰腺炎病变的有11例(92%),同时累及3个以上脏器的2例(16.7%)。2例治疗前接受胆管内支架置入术的患者均在激素使用46周后取出胆管内支...

     

  • [1]BRITO-ZERON P, RAMOS-CASALS M, BOSCH X, et al.The clinical spectrum of Ig G4-related disease[J].Autoimmun Rev, 2014, 13 (12) :1203-1210.
    [2]CULVER EL, CHAPMAN RW.Ig G4-related hepatobiliary disease:an overview[J].Nat Rev Gastroenterol Hepatol, 2016, 13 (10) :601-612.
    [3]OHARA H, OKAZAKI K, TSUBOUCHI H, et al.Clinical diagnostic criteria of Ig G4-related sclerosing cholangitis 2012[J].J Hepatobiliary Pancreat Sci, 2012, 19 (5) :536-542.
    [4]NAKAZAWA T, NAITOH I, OHARA T, et al.Diagnostic criteria for Ig G4-related sclerosing cholangitis based on cholangiographic classification[J].J Gastroenterol, 2012, 47 (1) :79-87.
    [5]KAMISAWA T, FUNATA, HAYASHI Y, et al.Lymphoplasmacytic sclerosing pancreatitis is a pancreatic lesion of Ig G4-related systemic[J].Am J Surg Pathol, 2004, 28 (8) :1114.
    [6]MARTINEZ-VALLE F, FEMANDEZ-CODINA A, PINAL-FEMANDEZ L, et al.Ig G4-related disease:Evidence from six recent cohorts[J].Autoimmun Rev, 2017, 16 (2) :168-172.
    [7]BJOENSSON E, CHARI ST, SMYRK TC, et al.Immunoglobulin G4 associated cholangitis:Description of an emerging clinical entity based on review of the literature[J].Hepatology, 2007, 45 (6) :1547-1554.
    [8]NAKAZAWA T, IKEDA Y, KAWAGUCHI Y, et al.Isolated intrapancreatic Ig G4-related sclerosing cholangitis[J].World J Gastroenterol, 2015, 21 (4) :1334-1343.
    [9]ATSUSHI K, ICHIRO T, ISAO N, et al.Nationwide epidemiological survey of autoimmune[J].Pancreas, 2015, 44 (4) :535-539.
    [10]INOUE D, YOSHIDA K, YONEDA N, et al.Ig G4-related disease:dataset of 235 consecutive patients[J].Medicine (Baltimore) , 2015, 94 (15) :e680.
    [11]ZEN Y, KAWAKAMI H, KIM JH, et al.Ig G4-related sclerosing cholangitis:all we need to know[J].J Gastroenterol, 2016, 51 (4) :295-312.
    [12]ZEN Y, FUJII T, HARADA K, et al.Th2 and regulatory immune reactions are increased in immunoglobin G4-related sclerosing pancreatitis and cholangitis[J].Hepatology, 2007, 45 (6) :1538-1546.
    [13]MIYOSHI H, UCHIDA K, TANIGUCHI T, et al.Circulating na6ve and CD4+CD25high regulatory T cells in patients with autoimmune pancreatitis[J].Pancreas, 2008, 36 (2) :133-140.
    [14]CARRUTHERS MN, KHOSROSHAHI A, AUGUSTIN T, et al.The diagnostic utility of serum Ig G4 concentrations in Ig G4-related disease[J].Ann Rheum Dis, 2015, 74 (1) :14-18.
    [15]BOONSTRA K, CULVER EL, POEN AC, et al.Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin G4-associated cholangitis from primary sclerosing cholangitis[J].Hepatology, 2014, 59 (5) :1954-1963.
    [16]CULVER EL, SADLER R, SIMPSON D, et al.Elevated serum Ig G4 levels in diagnosis, treatment response, organ involvement, and relapse in a prospective Ig G4-related disease UK cohort[J].Am J Gastroenterol, 2016, 111 (5) :733-743.
    [17]GHAZALE A, CHARI ST, ZHANG L, et al.Immunoglobulin G4-associated cholangitis:clinical profile and response to therapy[J].Gastroenterology, 2008, 134 (3) :706-715.
    [18]MASAMUNE A, NISHIMORI I, KIKUTA K, et al.Randomised controlled trial of long-term maintenance corticosteroid therapy in patients with autoimmune pancreatitis[J].Gut, 2017, 66 (3) :487-494.
    [19]YOU MW, KIM JH, BYUN JH, et al.Relapse of Ig G4-related sclerosing cholangitis after steroid therapy:Image findings and risk factors[J].Eur Radiol, 2014, 24 (5) :1039-1048.
    [20]CARRUTHERS MN, TOPAZIAN MD, KHOSROSHAHI A, et al.Rituximab for Ig G4-related disease:A prospective, open-label trial[J].Ann Rheum Dis, 2015, 74 (6) :1171-1177.
    [21]KHOSROSHAHI A, BLOCH DB, DESHPANDE V, et al.Rituximab therapy leads to rapid decline of serum Ig G4 levels and prompt clinical improvement in Ig G4-related systemic disease[J].Arthritis Rheum, 2010, 62 (6) :1755-1762.
    [22]KHOSROSHAHI A, CARRUTHERS MN, DESHPANDE V, et al.Rituximab for the treatment of Ig G4-related disease:Lessons from10 consecutive patients[J].Medicine (Baltimore) , 2012, 91 (1) :57-66.
    [23]MURAKAMI J, MATSUI S, ISHIZAWA S, et al.Recurrence of Ig G4-related disease following treatment with rituximab[J].Mod Rheumatol, 2013, 23 (6) :1226-1230.
  • 加载中
计量
  • 文章访问数:  1987
  • HTML全文浏览量:  28
  • PDF下载量:  389
  • 被引次数: 0
出版历程
  • 收稿日期:  2017-11-20
  • 出版日期:  2018-06-20
  • 分享
  • 用微信扫码二维码

    分享至好友和朋友圈

目录

    /

    返回文章
    返回