触须样免疫性肾小球病：1例报告并文献复习

doi:10.3877/cma.j.issn.2095-3216.2024.04.009

中华肾病研究电子杂志 ›› 2024, Vol. 13 ›› Issue (04) : 231 -233. doi: 10.3877/cma.j.issn.2095-3216.2024.04.009

病例报告

触须样免疫性肾小球病：1例报告并文献复习

吴爱华¹, 沐晓蝶¹, 杨敏¹, 邹芸¹, 周华¹^,^†(

)

^1. 213003　常州市第一人民医院肾内科

收稿日期:2023-04-06 出版日期:2024-08-28
通信作者: 周华
基金资助:
国家自然科学基金(82000684); 常州市"十四五"卫生健康高层次人才培养工程—拔尖人才([2022]109); 常州市科技局科技支撑（社会发展）项目(CE20215024)

Immunotactoid glomerulopathy: a case report and literature review

Aihua Wu, Xiaodie Mu, Min Yang

Received:2023-04-06 Published:2024-08-28

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触须样免疫性肾小球病(immunotactoid glomerulopathy, ITG)是一种罕见的肾脏疾病，国内外文献中报道的病例数不足100例^[1]。肾脏病理电镜观察可见特征性中空微管结构沉积在系膜区和基底膜内，少见肾外系统受累。其发病机制仍不清楚，可能与浆细胞或B淋巴细胞异常增殖相关，常继发于血液系统疾病^[2]。近年来的研究强调靶向浆细胞或B淋巴细胞治疗ITG，较少关注糖皮质激素对ITG的作用。本文报道1例ITG，探讨了本病的临床表现、病理特征、诊断和治疗等。

图1 肾组织病理检查结果注：A：系膜细胞和基质中重度增生(HE染色×200)；B：基底膜内皮下及袢腔内嗜复红物沉积(Masson染色×400)；C：IgG沿系膜区、内皮下、袢腔内团块状沉积(免疫荧光×400)；D：κ轻链沿系膜区、内皮下、袢腔内团块状沉积(免疫荧光×400)；E：系膜区、基底膜内呈束或平行排列的中空无分支微管状结构(电镜×10 000)；F：系膜区、基底膜内呈束或平行排列的中空无分支微管状结构，管状物直径约20～60 nm(电镜×20 000)

[1]	Javaugue V, Dufour-Nourigat L, Desport E, et al. Results of a nation-wide cohort study suggest favorable long-term outcomes of clone-targeted chemotherapy in immunotactoid glomerulopathy [J]. Kidney Int, 2021, 99(2): 421-430.
[2]	Nasr SH, Kudose SS, Said SM, et al. Immunotactoid glomerulopathy is a rare entity with monoclonal and polyclonal variants [J]. Kidney Int, 2021, 99(2): 410-420.
[3]	Fogo AB, Lusco MA, Najafian B, et al. AJKD atlas of renal pathology: immunotactoid glomerulopathy [J]. Am J Kidney Dis, 2015, 66(4): e29-e30.
[4]	Carrara C, Ferucci E, Emili S, et al. Immunotactoid glomerulopathy of 10-years′ duration: insights gained from sequential biopsies [J]. Kidney Int Rep, 2017, 2(5): 978-983.
[5]	Leung N, Bridoux F, Batuman V, et al. Publisher correction: the evaluation of monoclonal gammopathy of renal significance : a consensus report of the International Kidney and Monoclonal Gammopathy Research Group [J]. Nat Rev Nephrol, 2019, 15(2): 121.
[6]	Ohashi A, Kumagai J, Nagahama K, et al. Case of immunotactoid glomerulopathy showing high responsiveness to steroids therapy despite severe pathological features [J]. BMJ Case Rep, 2019, 12(7) : e229751.
[7]	Kinomura M, Maeshima Y, Kodera R, et al. A case of immunotactoid glomerulopathy exhibiting nephrotic syndrome successfully treated with corticosteroids and antihypertensive therapy[J]. Clin Exp Nephrol, 2009, 13(4): 378-384.

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