Coexistence of Anti-Glomerular Basement Membrane Antibodies and Anti-Neutrophil Cytoplasmic Antibodies in a Child With Human Leukocyte Antigen Susceptibility and Detailed Antibody Description: A Case Report.
Coexistence of Anti-Glomerular Basement Membrane Antibodies and Anti-Neutrophil Cytoplasmic Antibodies in a Child With Human Leukocyte Antigen Susceptibility and Detailed Antibody Description: A Case Report.
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人类白细胞抗原易感儿童抗肾小球基底膜抗体与抗中性粒细胞胞浆抗体共存及抗体详细描述一例报告
DOI：
10.1097/md.0000000000001179
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发表时间：
2015-07
期刊：
影响因子：
1.6
通讯作者：
Zhao MH
中科院分区：
文献类型：
--
作者：
Xie LJ;Cui Z;Jia XY;Chen Z;Liu XR;Zhao MH
抗肾小球基底膜（抗GBM）疾病和抗中性粒细胞胞浆抗体（ANCA）相关血管炎均可能导致快速进展性肾小球肾炎。 ANCA 和抗 GBM 抗体同时存在被称为“双阳性”，这在儿童中极为罕见。我们报告了一例 ANCA 和抗 GBM 抗体共存的儿科病例。一名6岁女孩出现急性肾功能衰竭、血尿、蛋白尿、少尿。她的髓过氧化物酶特异性 ANCA 和抗 GBM 抗体呈双阳性。肾脏活检证实沿 GBM 存在线性免疫球蛋白 (Ig)G 沉积，并且肾小球中 100% 形成新月体；其中细胞新月体占83.3%。人类白细胞抗原 (HLA) 基因分型显示 DRB1*1501（一种与抗 GBM 疾病密切相关的等位基因）和 DRB1*0405（ANCA 相关性血管炎患者肾衰竭的独立危险因素）。抗GBM抗体效价为1:800，主要IgG亚型为IgG1，与严重肾损伤和较差预后密切相关。抗 GBM 抗体的靶抗原限制在 IV 型胶原 (α3[IV]NC1) 的 α3 链的非胶原结构域 1 上，可识别 EA (α317–31) 和 EB (α3127–141) 这两个表位。这是首例报道的ANCA和抗GBM抗体共存的儿科病例，其中鉴定了自身抗体的HLA分型和免疫学特征。该早发患者的研究结果对于了解抗 GBM 疾病和 ANCA 相关血管炎的机制具有重要意义。
Anti-glomerular basement membrane (anti-GBM) disease and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis both could cause rapidly progressive glomerulonephritis. The coexistence of ANCAs and anti-GBM antibodies was known as “double positive,” which was extremely rare in children. We report a pediatric case with coexistence of ANCAs and anti-GBM antibodies. A 6-year-old girl presented with acute renal failure, hematuria, proteinuria, and oliguria. She was double positive of ANCAs specific to myeloperoxidase, and anti-GBM antibodies. Kidney biopsy confirmed linear immunoglobulin (Ig)G deposit along GBM and 100% of crescent formation in glomeruli; among them 83.3% were cellular crescents. Human leukocyte antigen (HLA) gene typing showed DRB1∗1501, an allele strongly associated with anti-GBM disease, and DRB1∗0405, an independent risk factor for renal failure in patients with ANCA-associated vasculitis. The titer of anti-GBM antibodies was 1:800, and the predominant IgG subclass was IgG1, which was closely related with severe kidney injury and worse outcome. The target antigen of anti-GBM antibodies was restricted on the noncollagen domain 1 of the α3 chain of type IV collagen (α3[IV]NC1), with recognitions to both epitopes, EA (α317–31) and EB (α3127–141). This is the first reported pediatric case with coexistence of ANCAs and anti-GBM antibodies, in which the HLA typing and immunologic characters of autoantibodies were identified. The findings on this early-onset patient are meaningful for understanding the mechanisms of both anti-GBM disease and ANCA-associated vasculitis.