切换至 "中华医学电子期刊资源库"

中华肾病研究电子杂志 ›› 2024, Vol. 13 ›› Issue (03) : 177 -179. doi: 10.3877/cma.j.issn.2095-3216.2024.03.009

病例报告

膜性肾病合并ANCA相关性血管炎:1例报告及文献复习
杨飞1, 王小龙2, 吴杰2,(), 陈婧婧2, 陈仆2, 尹忠2, 蔡广研2   
  1. 1. 564500 仁怀市人民医院
    2. 100853 北京,解放军总医院第一医学中心肾脏病医学部、肾脏疾病国家重点实验室、国家慢性肾病临床医学研究中心、肾脏疾病研究北京市重点实验室
  • 收稿日期:2022-10-25 出版日期:2024-06-28
  • 通信作者: 吴杰

Membranous nephropathy complicated with ANCA-associated vasculitis: a case report with literature review

Fei Yang, Xiaolong Wang, Jie Wu()   

  • Received:2022-10-25 Published:2024-06-28
  • Corresponding author: Jie Wu
引用本文:

杨飞, 王小龙, 吴杰, 陈婧婧, 陈仆, 尹忠, 蔡广研. 膜性肾病合并ANCA相关性血管炎:1例报告及文献复习[J]. 中华肾病研究电子杂志, 2024, 13(03): 177-179.

Fei Yang, Xiaolong Wang, Jie Wu. Membranous nephropathy complicated with ANCA-associated vasculitis: a case report with literature review[J]. Chinese Journal of Kidney Disease Investigation(Electronic Edition), 2024, 13(03): 177-179.

膜性肾病(membranous nephropathy,MN)是成人肾病综合征的主要病因,它的免疫病理特征是上皮下免疫球蛋白(IgG)和补体(C3)呈颗粒样沉积,导致肾小球基底膜(GBM)的改变[1]。通过光学显微镜可观察到毛细血管壁增厚。大多数患者以肾病综合征起病,约20%的患者表现为无症状、非肾病范围的蛋白尿。在发达国家,75%MN为特发性膜性肾病(idiopathic membranous nephropathy,IMN),其余则为继发性膜性肾病,如继发于感染、系统性自身免疫病、药物和恶性肿瘤等[2]。抗中性粒细胞胞质抗体相关性血管炎(anti-neutrophil cytoplasmic antibody-associated vasculitis,AAV)是以坏死性小血管炎为特征的一组免疫性疾病[3],主要包括显微镜下多血管炎、肉芽肿性多血管炎、嗜酸性肉芽肿性多血管炎;可累及全身多个系统器官,其中肾脏受累最常见。MN合并AAV非常罕见,本文报道1例。

图1 肾脏活检病理组织学注:A:肾小球见局灶、节段、轻度系膜细胞增殖及系膜基质增宽,内皮细胞未见明显增殖,毛细血管袢开放尚可,包曼氏囊壁节段增厚、分层;可见肾小管萎缩,部分肾小管刷状缘、上皮细胞脱落、上皮细胞再生重排;间质见灶性炎细胞浸润,炎细胞以淋巴/单核细胞为主,灶性纤维化(PAS染色×100);B:肾内小动脉壁见玻璃样变性,小动脉周围见炎细胞包绕,炎细胞以淋巴/单核细胞为主(PAS染色×400);C:肾内小动脉壁见玻璃样变性,小动脉周围见炎细胞包绕,炎细胞以淋巴/单核细胞为主(PASM染色×100);D(PASM染色×100)和E(PASM染色×400):肾小球基底膜明显增厚,上皮下可见大量嗜复红物质沉积,基底膜可见空泡、钉突及链环状改变;F:免疫荧光(×200)可见:IgG(2+)、IgM(1+)、C3(1+)、Fib(1+)、IgG1(1+)、IgG4(2+)沿毛细血管袢颗粒样沉积,IgA、C4和C1q均阴性
[1]
Zou R, Liu G, Cui Z, et al. Clinical and immunologic characteristics of patients with ANCA-associated glomerulonephritis combined with membranous nephropathy: a retrospective cohort study in a single Chinese center [J]. Medicine (Baltimore), 2015, 94(37): e1472.
[2]
von Haxthausen F, Reinhard L, Pinnschmidt HO, et al. Antigen-specific IgG subclasses in primary and malignancy-associated membranous nephropathy [J]. Front Immunol, 2018, 9: 3035.
[3]
谢丽娇,张瑞,贺江花,等. ANCA相关性血管炎伴血清补体C3降低的临床分析[J]. 免疫学杂志2017, 33(5): 410-414, 421.
[4]
Gaber LW, Wall BM, Cooke CR. Coexistence of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis and membranous glomerulopathy [J]. Am J Clin Pathol, 1993, 99(2): 211-215.
[5]
欧海姿. 合并膜性肾病样病理改变的ANCA相关性肾小球肾炎临床和病理分析——单中心回顾性研究[D]. 南宁:广西医科大学,2019.
[6]
Tse WY, Howie AJ, Adu D, et al. Association of vasculitic glomerulonephritis with membranous nephropathy: a report of 10 cases [J]. Nephrol Dial Transplant, 1997, 12(5): 1017-1027.
[7]
Nasr SH, Said SM, Valeri AM, et al. Membranous glomerulonephritis with ANCA-associated necrotizing and crescentic glomerulonephritis [J]. Clin J Am Soc Nephrol, 2009, 4(2): 299-308.
[8]
Hanamura K, Tojo A, Kinugasa S, et al. Detection of myeloperoxidase in membranous nephropathy-like deposits in patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis [J]. Hum Pathol, 2011, 42(5): 649-658.
[9]
Manabe S, Hatano M, Nakano M, et al. Myeloperoxidase-anti-neutrophil cytoplasmic antibody causes different renal diseases by immune-complex formation and pauci-immune mechanism: a case report [J]. Pathol Int, 2017, 67(8): 419-424.
[10]
Kanahara K, Yorioka N, Nakamura C, et al. Myeloperoxidase-antineutrophil cytoplasmic antibody-associated glomerulonephritis with membranous nephropathy in remission [J]. Intern Med, 1997, 36(11): 841-846.
No related articles found!
阅读次数
全文


摘要