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中华肾病研究电子杂志 ›› 2022, Vol. 11 ›› Issue (05) : 294 -296. doi: 10.3877/cma.j.issn.2095-3216.2022.05.010

病例报告

IgG4相关疾病累及多系统1例及文献复习
王耀巍1, 杨丛旭1, 余仁欢1,()   
  1. 1. 100091 北京,中国中医科学院西苑医院肾病科
  • 收稿日期:2022-03-29 出版日期:2022-10-28
  • 通信作者: 余仁欢
  • 基金资助:
    中国中医科学院科技创新工程(CI2021A01208)

A case of IgG4-related disease involving multiple systems and literature review

Yaowei Wang1, Congxu Yang1, Renhuan Yu1()   

  • Received:2022-03-29 Published:2022-10-28
  • Corresponding author: Renhuan Yu
引用本文:

王耀巍, 杨丛旭, 余仁欢. IgG4相关疾病累及多系统1例及文献复习[J]. 中华肾病研究电子杂志, 2022, 11(05): 294-296.

Yaowei Wang, Congxu Yang, Renhuan Yu. A case of IgG4-related disease involving multiple systems and literature review[J]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2022, 11(05): 294-296.

IgG4相关疾病(IgG4-related disease,IgG4-RD)是由免疫介导的慢性进行性炎症纤维化疾病,可累及多器官或组织,目前其发病机制尚不明确。IgG4-RD临床特点包括:(1)血清学特征:血清IgG4水平升高;(2)影像学特征:受累器官出现局限或弥漫性肿大、结节、肿块、壁增厚;(3)病理特征:受累组织出现淋巴细胞、浆细胞浸润,其中以IgG4+浆细胞为主,并常伴有席纹状纤维化病理改变。因临床表现无特异性,诊治难度较大。本文报道1例因"肾功能不全"就诊的患者,既往病史14年,反复因器官肿块、结节就医,最终于我院确诊为IgG4-RD。

图1 淋巴结组织形态学观测注:A:淋巴结生发中心部位萎缩,副皮质区内浆细胞增生明显伴少量嗜酸性粒细胞[苏木精-伊红(hematoxylin and eosin,HE)×200];B:CD138浆细胞成棕黄色[免疫组织化学(immunohistochemistry,IHC)×200];C:IgG阳性的浆细胞数量明显增多(IHC×200); D:IgG4阳性细胞数量明显增多(IHC×200)
表1 患者2007年至2022年基本情况及治疗后随访情况
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