Circulating Anti-Glomerular Basement Membrane Antibodies With Predominance of Subclass IgG4 and False-Negative Immunoassay Test Results in Anti-Glomerular Basement Membrane Disease
Circulating Anti-Glomerular Basement Membrane Antibodies With Predominance of Subclass IgG4 and False-Negative Immunoassay Test Results in Anti-Glomerular Basement Membrane Disease
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DOI：
10.1053/j.ajkd.2013.08.032
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发表时间：
2014-02-01
影响因子：
13.2
通讯作者：
Segelmark, Marten
中科院分区：
文献类型：
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作者：
Ohlsson, Sophie;Herlitz, Hans;Segelmark, Marten
抗肾小球基底膜（GBM）成分（IV型胶原的α 3链）的自身抗体可引起快速进行性肾小球肾炎和肺泡出血，称为抗GBM疾病或Goodpasture疾病。抗GBM抗体通常属于免疫球蛋白G亚类1（IgG1），在大多数情况下，可以使用酶联免疫吸附测定（ELISA）在循环中容易地检测到。我们报告4例抗GBM ELISA阴性或临界结果，尽管危及生命的疾病。4例患者IgG 4抗GBM ELISA检测均为阳性，抗中性粒细胞胞浆抗体均未检出。所有病例均经肾活检证实。当使用非变性包被缓冲液时，两名患者在抗GBM ELISA中显示出更高的信号。所有4例患者均为年轻女性，伴有严重肺泡出血和良好的肾脏结局，提示IgG4自身抗体占优势的患者可能构成抗GBM疾病的一个独特亚组。我们的结论是，特发性肺泡出血的患者可以有抗GBM疾病检测只有IgG亚类特异性试验或肾活检。(C)2014年由国家肾脏基金会，Inc.
Autoantibodies against a constituent of the glomerular basement membrane (GBM), the alpha 3-chain of type IV collagen, can cause both rapidly progressive glomerulonephritis and alveolar hemorrhage, referred to as anti-GBM disease or Goodpasture disease. Anti-GBM antibodies generally are of immunoglobulin G subclass 1 (IgG1) and can in most cases readily be detected in the circulation using enzyme-linked immunosorbent assays (ELISAs). We report 4 cases in which anti-GBM ELISA yielded negative or borderline results despite life-threatening disease. All 4 patients had positive results by IgG4 anti-GBM ELISA and all had undetectable anti-neutrophil cytoplasmic antibody. All cases were confirmed with kidney biopsy. Two of the patients showed higher signal in anti-GBM ELISA when using a nondenaturing coating buffer. All 4 were young women with severe alveolar hemorrhage and favorable renal outcome, suggesting that patients with predominance of IgG4 autoantibodies may constitute a distinct subgroup of anti-GBM disease. We conclude that patients with idiopathic alveolar hemorrhage can have anti-GBM disease detected by only IgG subclass-specific tests or kidney biopsy. (C) 2014 by the National Kidney Foundation, Inc.