Utility of glomerular Gd-IgA1 staining for indistinguishable cases of IgA nephropathy or Alport syndrome
Utility of glomerular Gd-IgA1 staining for indistinguishable cases of IgA nephropathy or Alport syndrome
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DOI：
10.1007/s10157-021-02054-3
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发表时间：
2021-03
影响因子：
2.3
通讯作者：
Shinya Ishiko;A. Tanaka;A. Takeda;M. Hara;Naoto Hamano;M. Koizumi;T. Ueno;H. Hayashi;Atsushi Ko
中科院分区：
文献类型：
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作者：
Shinya Ishiko;A. Tanaka;A. Takeda;M. Hara;Naoto Hamano;M. Koizumi;T. Ueno;H. Hayashi;Atsushi Ko
研究背景Alport综合征的病理表现常表现为系膜增生，有时还伴有IgA沉积，此外还有独特的肾小球基底膜(GBM)改变，包括基底膜变薄和/或板层。然而，类似的基底膜异常也经常在IgA肾病中观察到。众所周知，这两种疾病也会出现血尿、蛋白尿，有时还会出现大量血尿，当与病毒感染有关时。因此，即使根据临床和病理结果，也很难做出鉴别诊断。方法对5例既有IgA沉积又有肾小球基底膜改变的患者进行基因筛查和肾小球Gd-Ig A1及IV型胶原α5链免疫组织化学染色，以确定Gd-Ig A1有助于鉴别这两种疾病。在例1-4中，未检测到肾小球Gd-IgA1沉积，尽管在系膜区有IgA阳性。例5可见肾小球Gd-IgA1沉积。结论Gd-IgA1表达分析可明确鉴别这两种疾病。这种方法可以用来识别这两种临床和病理结果相同的疾病。
BackgroundPathological findings in Alport syndrome frequently show mesangial proliferation and sometimes incidental IgA deposition, in addition to unique glomerular basement membrane (GBM) changes including thin basement membrane and/or lamellation. However, similar GBM abnormalities are also often observed in IgA nephropathy. Both diseases are also known to show hematuria, proteinuria, and sometimes macrohematuria when associated with viral infection. Therefore, it can be difficult to make a differential diagnosis, even based on clinical and pathological findings. Some recent articles demonstrated that galactose-deficient IgA1 (Gd-IgA1)-specific monoclonal antibody (KM55) could potentially enable incidental IgA deposition to be distinguished from IgA nephropathy.MethodsWe performed comprehensive gene screening and glomerular Gd-IgA1 and type IV collagen α5 chain immunostaining for five cases with both IgA deposition and GBM changes to confirm that Gd-IgA1 can help to distinguish these two diseases.ResultsFour of the cases were genetically diagnosed with Alport syndrome (Cases 1–4) and one was IgA nephropathy with massive GBM changes, which had a negative gene test result (Case 5). In Cases 1–4, glomerular Gd-IgA1 deposition was not detected, although there was positivity for IgA in the mesangial area. In Case 5, glomerular Gd-IgA1 deposition was observed.ConclusionGd-IgA1 expression analysis could clearly differentiate these two disorders. This approach can be applied to identify these two diseases showing identical clinical and pathological findings.