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膜性或膜样肾小球肾炎：一例大量蛋白尿、血清学阳性但活检时PLA2R阴性的病例报告

Membranous or membranous-like GN: A case report of massive proteinuria, positive serum with negative PLA2R on biopsy.

作者信息

Kaur Gurwant, Chen Guoli

机构信息

Department of Medicine (Nephrology) Penn State Milton S. Hershey Medical Centre Hershey Pennsylvania.

Department of Pathology Penn State Milton S. Hershey Medical Centre Hershey Pennsylvania.

出版信息

Clin Case Rep. 2018 Oct 9;6(11):2198-2201. doi: 10.1002/ccr3.1849. eCollection 2018 Nov.

DOI:10.1002/ccr3.1849
PMID:30455920
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6230598/
Abstract

This case report represents primary membranous glomerulonephritis (MGN) with positive serum anti-PLA2R antibodies, 2+ positivity for IgG4 on immunofluorescence with routine fresh-frozen sections and negative PLA2R stain on biopsy. He was treated as primary MGN based on positive serum PLA2R and the absence of clinical symptoms or signs suggestive of any secondary MGN.

摘要

本病例报告为一例原发性膜性肾小球肾炎（MGN），血清抗磷脂酶A2受体（PLA2R）抗体阳性，常规新鲜冰冻切片免疫荧光显示IgG4呈2+阳性，活检时PLA2R染色阴性。基于血清PLA2R阳性且无提示任何继发性MGN的临床症状或体征，该患者被诊断为原发性MGN并接受治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/f2dea8d5a678/CCR3-6-2198-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/c38cd41d828e/CCR3-6-2198-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/3b5c9ae7e3e0/CCR3-6-2198-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/7c47af07e3bd/CCR3-6-2198-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/f2dea8d5a678/CCR3-6-2198-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/c38cd41d828e/CCR3-6-2198-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/3b5c9ae7e3e0/CCR3-6-2198-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/7c47af07e3bd/CCR3-6-2198-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b571/6230598/f2dea8d5a678/CCR3-6-2198-g004.jpg

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本文引用的文献

1
Clinicopathologic Features of Membranous-Like Glomerulopathy With Masked IgG Kappa Deposits.伴有隐匿性IgG κ轻链沉积的膜样肾小球病的临床病理特征
Kidney Int Rep. 2016 Aug 24;1(4):299-305. doi: 10.1016/j.ekir.2016.08.012. eCollection 2016 Nov.
2
Diagnostic specificity of autoantibodies to M-type phospholipase A2 receptor (PLA2R) in differentiating idiopathic membranous nephropathy (IMN) from secondary forms and other glomerular diseases.抗 M 型磷脂酶 A2 受体 (PLA2R) 自身抗体在鉴别特发性膜性肾病 (IMN) 与继发性及其他肾小球疾病中的诊断特异性。
J Nephrol. 2018 Apr;31(2):271-278. doi: 10.1007/s40620-017-0451-5. Epub 2017 Oct 28.
3
Pathophysiological advances in membranous nephropathy: time for a shift in patient's care.
膜性肾病的病理生理学进展：是时候改变患者的治疗方法了。
Lancet. 2015 May 16;385(9981):1983-92. doi: 10.1016/S0140-6736(15)60731-0.
4
Membranous-like glomerulopathy with masked IgG kappa deposits.膜性样肾小球病伴隐匿性 IgGκ 沉积。
Kidney Int. 2014 Jul;86(1):154-61. doi: 10.1038/ki.2013.548. Epub 2014 Jan 15.
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Differential diagnosis of membranous nephropathy with autoantibodies to phospholipase A2 receptor 1.抗磷脂酶 A2 受体 1 自身抗体所致膜性肾病的鉴别诊断。
Autoimmun Rev. 2014 Feb;13(2):108-13. doi: 10.1016/j.autrev.2013.09.005. Epub 2013 Sep 26.
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Determination of primary versus secondary membranous glomerulopathy utilizing phospholipase A2 receptor staining in renal biopsies.利用肾活检中磷酸酶 A2 受体染色鉴定原发性与继发性膜性肾小球病。
Mod Pathol. 2013 May;26(5):709-15. doi: 10.1038/modpathol.2012.207. Epub 2012 Nov 30.
7
Membranous nephropathy (bubbling appearance and spike formation) without immunoglobulin deposition in a patient with systemic lupus erythematosus.系统性红斑狼疮患者的膜性肾病（呈泡状外观和钉突形成），无免疫球蛋白沉积。
Clin Exp Nephrol. 2008 Dec;12(6):475-8. doi: 10.1007/s10157-008-0096-8. Epub 2008 Oct 7.
8
Immunoperoxidase versus immunofluorescence in the assessment of human renal biopsies.免疫过氧化物酶法与免疫荧光法在人肾活检评估中的应用
Am J Kidney Dis. 2005 Apr;45(4):674-83. doi: 10.1053/j.ajkd.2004.12.019.