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多部位病理证实的IgG4相关性疾病一例

汪劭婷 孙雪峰

汪劭婷, 孙雪峰. 多部位病理证实的IgG4相关性疾病一例[J]. 协和医学杂志, 2017, 8(4-5): 274-277. doi: 10.3969/j.issn.1674-9081.2017.05.015
引用本文: 汪劭婷, 孙雪峰. 多部位病理证实的IgG4相关性疾病一例[J]. 协和医学杂志, 2017, 8(4-5): 274-277. doi: 10.3969/j.issn.1674-9081.2017.05.015
Shao-ting WANG, Xue-feng SUN. A Case of IgG4-related Disease Confirmed by Pathology with Multiple Lesions[J]. Medical Journal of Peking Union Medical College Hospital, 2017, 8(4-5): 274-277. doi: 10.3969/j.issn.1674-9081.2017.05.015
Citation: Shao-ting WANG, Xue-feng SUN. A Case of IgG4-related Disease Confirmed by Pathology with Multiple Lesions[J]. Medical Journal of Peking Union Medical College Hospital, 2017, 8(4-5): 274-277. doi: 10.3969/j.issn.1674-9081.2017.05.015

多部位病理证实的IgG4相关性疾病一例

doi: 10.3969/j.issn.1674-9081.2017.05.015
详细信息
    通讯作者:

    孙雪峰  电话:010-69155154,E-mail:sunxfer@sina.com

  • 中图分类号: R563.9

A Case of IgG4-related Disease Confirmed by Pathology with Multiple Lesions

More Information
    Corresponding author: SUN Xue-feng  Tel: 010-69155154,E-mail:sunxfer@sina.com
  • 摘要: IgG4相关性疾病(IgG4-related disease, IgG4-RD)是累及多器官或组织的慢性进行性自身免疫性疾病, 其病理为受累脏器被淋巴细胞和IgG4阳性的浆细胞浸润, 肺部表现多样。本文报道了一例以咳嗽、气短起病的IgG4-RD, 有肺、纵膈淋巴结、左颌下淋巴结、腹膜后、脾门、胃粘膜、皮肤受累, 其肺野内病变表现为弥漫微结节, 激素治疗后完全消失。
  • 图  1  肺CT示双肺间质纹理增厚,弥漫分布小结节;两肺门、纵隔及双腋窝多发肿大淋巴结;双肺多发斑片影及磨玻璃密度影,局部可见牵拉性支气管扩张

    图  2  头颈增强CT示左侧颌下及双侧颈动脉鞘区多发淋巴结影,部分增大,右侧面颊软组织增厚,轻度均匀强化,左侧颌下腺受压推挤

    图  3  左颌下淋巴结病理示广泛浆细胞浸润,结合免疫组织化学病变符合IgG4相关性淋巴结病(HE, ×10)

    图  4  经激素治疗1个月后复查肺CT示肺内病变基本消失

  • [1] Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease(IgG4-RD)[J]. Mod Rheumatol, 2012, 22:21-30. doi:  10.3109/s10165-011-0571-z
    [2] Geyer JT, Niesvizky R, Jayabalan DS, et al.IgG4 plasma cell myeloma:new insights into the pathogenesis of IgG4-related disease[J].Mod Pathol, 2014, 27:375-381. doi:  10.1038/modpathol.2013.159
    [3] Inoue D, Yoshida K, Yoneda N, et al. IgG4-related disease:dataset of 235 consecutive patients[J].Medicine(Baltimore), 2015, 94:e680. http://cn.bing.com/academic/profile?id=8a4b6cb03b1b854b23f3d478307cca48&encoded=0&v=paper_preview&mkt=zh-cn
    [4] Inoue D, Zen Y, Abo H, et al. Immunoglobulin G4-related lung disease:CT findings with pathologic correlations[J]. Radiology, 2009, 251:260-70. doi:  10.1148/radiol.2511080965
    [5] Matsui S, Hebisawa A, Sakai F, et al. Immunoglobulin G4-related lung disease:Clinicoradiological and pathological features[J]. Respirology, 2013, 18:480-487. doi:  10.1111/resp.12016
    [6] Suzuki H, Watanabe M, Ara T, et al. Immunoglobulin G4-related lung disease accompanied by organizing pneumonia[J].Intern Med, 2013, 52:2105-2111. doi:  10.2169/internalmedicine.52.0112
    [7] Duvic C, Desrame J, Lévêque C, et al.Retroperitoneal fibrosis, sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia[J]. Nephrol Dial Transplant, 2004, 19:2397-2399. doi:  10.1093/ndt/gfh050
    [8] Onishi Y, Kawamura T, Kagami R, et al.IgG4-related lung disease with organizing pneumonia effectively treated with azathioprine[J].Intern Med, 2014, 53:2701-2704. doi:  10.2169/internalmedicine.53.2564
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出版历程
  • 收稿日期:  2015-12-17
  • 刊出日期:  2017-09-30

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