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首页> 外文期刊>CEN Case Reports >Proliferative glomerulonephritis with monoclonal IgG2κ deposit successfully treated with steroids: a case report and review of the literature
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Proliferative glomerulonephritis with monoclonal IgG2κ deposit successfully treated with steroids: a case report and review of the literature

机译：类固醇成功治疗增生性肾小球肾炎合并单克隆IgG2κ沉积：1例病例并文献复习

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摘要

A novel form of glomerular injury with monoclonal immunoglobulin (Ig) IgG deposition, termed “proliferative glomerulonephritis (GN) with monoclonal IgG deposits” (PGNMID), is a recently described entity. PGNMID presents with various histological patterns, such as membranoproliferative GN, endocapillary proliferative GN and membranous nephropathy (MN). The deposits are composed of monoclonal immunoglobulin, most commonly IgG3 and occasionally IgG2. At present, the clinical significance of each IgG subclass and the morphological patterns of glomerular injury have not been fully investigated due to the limited number of PGNMID cases reported. The patient was a 27-year-old woman presenting with a mild degree of proteinuria and no other physical or serological abnormalities. Monoclonal Ig could not be identified in her serum or urine. Renal biopsy found features of MN with deposition of monoclonal IgG2κ. Electron microscopy examination revealed non-organised electron-dense deposits predominantly in subepithelial locations. Based on a diagnosis of PGNMID, she was treated with prednisolone and proteinuria significantly decreased in less than 4 weeks. Although the clinical outcomes of PGNMID remain to be defined, MN features may possibly be a sign of favourable prognosis—a hypothesis supported by recent reports. The absence of advanced chronic damage in the kidney, such as glomerulosclerosis or tubulointerstitial fibrosis, may also have contributed to the favourable outcome in the present case. Further studies on additional PGNMID cases that allow the correlation of morphological features and IgG subclasses with clinical outcomes are needed in order to confirm our findings and further solidify the clinical aspects of this new disease entity.
机译：最近描述了一种具有单克隆免疫球蛋白（Ig）IgG沉积的新型肾小球损伤形式，称为“具有单克隆IgG沉积物的增生性肾小球肾炎（GN）”（PGNMID）。 PGNMID具有多种组织学模式，例如膜增生性GN，毛细血管内增生性GN和膜性肾病（MN）。沉积物由单克隆免疫球蛋白，最常见的IgG3和偶尔的IgG2组成。目前，由于报道的PGNMID病例数量有限，每个IgG亚类的临床意义和肾小球损伤的形态学模式尚未得到充分研究。该患者是一名27岁的女性，表现为轻度蛋白尿，无其他身体或血清学异常。在她的血清或尿液中无法鉴定出单克隆Ig。肾脏活检发现MN具有单克隆IgG2κ沉积的特征。电子显微镜检查发现无组织的电子致密沉积物主要在上皮下位置。根据PGNMID的诊断，她接受泼尼松龙治疗，蛋白尿在不到4周的时间内显着下降。尽管PGNMID的临床结局尚待确定，但MN的特征可能是预后良好的标志，这一假说得到了最近报道的支持。肾中不存在晚期慢性损害，例如肾小球硬化或肾小管间质纤维化，也可能有助于本病例的良好结果。为了证实我们的发现并进一步巩固这种新疾病实体的临床意义，需要进一步研究允许形态特征和IgG亚类与临床结果相关的其他PGNMID病例。

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  • 来源
    《CEN Case Reports》 |2013年第2期|197-203|共7页
  • 作者

    Ryuji Ohashi; Yukinao Sakai; Tomoyuki Otsuka; Dai Ohno; Yukinari Masuda; Tsuneo Murasawa; Naoki Sato; Akira Shimizu;

  • 作者单位

    Division of Diagnostic Pathology Nippon Medical School Hospital">(1);

    Division of Nephrology Department of Internal Medicine Nippon Medical School Musashikosugi Hospital">(2);

    Division of Nephrology Department of Internal Medicine Nippon Medical School Musashikosugi Hospital">(2);

    Division of Nephrology Department of Internal Medicine Nippon Medical School Musashikosugi Hospital">(2);

    Department of Pathology Nippon Medical School">(4);

    Division of Nephrology Department of Internal Medicine Nippon Medical School Musashikosugi Hospital">(2);

    Division of Cardiology Department of Internal Medicine and Intensive Care Medicine Nippon Medical School Musashikosugi Hospital">(3);

    Department of Pathology Nippon Medical School">(4);

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  • 关键词

    Proliferative glomerulonephritis; Monoclonal IgG; Membranous nephropathy; Steroid;

    机译：增生性肾小球肾炎;单克隆IgG;膜性肾病类固醇;

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