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血清抗肾小球基底膜(GBM)抗体阳性但IgG沉积阴性的抗GBM病:一例报告

Anti-GBM disease with positive serum anti-GBM antibodies but negative IgG deposition: A case report.

作者信息

Kazemzadeh Kimia, Kabiri Naeini Elham, Moeinzadeh Firouzeh, Naimi Azar

机构信息

Students' Scientific Research Center, Tehran University of Medical Sciences, Tehran, Iran.

Interest Group Department, Universal Scientific Education and Research Network, Tehran, Iran.

出版信息

SAGE Open Med Case Rep. 2023 Jul 31;11:2050313X231189767. doi: 10.1177/2050313X231189767. eCollection 2023.

Abstract

Anti-glomerular basement membrane antibodies are significantly specific for detecting anti-glomerular basement membrane disease. These antibodies are typically targeted against the non-collagenous (NC1) domain of the alpha 3 chain of type IV collagen and, to a lesser extent, the α4(IV) or α5(IV) chains, which create a triple-helical structure in the glomerular basement membrane. The modification of the hexameric structure of NC1 (α3(IV)) results in the exposure of new epitopes, leading to an immune reaction and the subsequent deposition of linear antibodies along the glomerular basement membrane, culminating in crescentic glomerulonephritis. Anti-glomerular basement membrane antibodies that are positive are believed to be pathogenic and capable of binding to the glomerular basement membrane in vivo, particularly in the context of rapidly progressive glomerulonephritis. Herein, we present a patient with positive serum anti-glomerular basement membrane antibodies but negative IgG deposition. The current findings are significant for raising physicians' awareness of the probable errors in detecting anti-glomerular basement membrane antibody disease as a possible cause of irreversible kidney failure.

摘要

抗肾小球基底膜抗体对于检测抗肾小球基底膜病具有显著特异性。这些抗体通常靶向IV型胶原α3链的非胶原(NC1)结构域,在较小程度上也靶向α4(IV)或α5(IV)链,它们在肾小球基底膜中形成三螺旋结构。NC1(α3(IV))六聚体结构的改变导致新表位暴露,引发免疫反应,随后线性抗体沿肾小球基底膜沉积,最终导致新月体性肾小球肾炎。阳性的抗肾小球基底膜抗体被认为具有致病性,能够在体内与肾小球基底膜结合,尤其是在快速进展性肾小球肾炎的情况下。在此,我们报告一名血清抗肾小球基底膜抗体阳性但IgG沉积阴性的患者。目前的发现对于提高医生对检测抗肾小球基底膜抗体疾病可能存在的错误的认识具有重要意义,因为这可能是不可逆肾衰竭的一个原因。

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