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类固醇成功治疗伴单克隆IgG2κ沉积的增殖性肾小球肾炎:一例报告并文献复习

Proliferative glomerulonephritis with monoclonal IgG2κ deposit successfully treated with steroids: a case report and review of the literature.

作者信息

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

机构信息

Division of Diagnostic Pathology, Nippon Medical School Hospital, 1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113-8603, Japan.

Division of Nephrology, Department of Internal Medicine, Nippon Medical School Musashikosugi Hospital, Kawasaki, Japan.

出版信息

CEN Case Rep. 2013 Nov;2(2):197-203. doi: 10.1007/s13730-013-0064-3. Epub 2013 Feb 26.

Abstract

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.

摘要

一种新型的肾小球损伤,伴有单克隆免疫球蛋白(Ig)IgG沉积,称为“伴有单克隆IgG沉积的增殖性肾小球肾炎(GN)”(PGNMID),是最近才被描述的一种疾病。PGNMID呈现出多种组织学模式,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组成,最常见的是IgG3,偶尔也有IgG2。目前,由于报道的PGNMID病例数量有限,每种IgG亚类的临床意义以及肾小球损伤的形态学模式尚未得到充分研究。该患者为一名27岁女性,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未检测到单克隆Ig。肾活检发现具有单克隆IgG2κ沉积的MN特征。电子显微镜检查显示主要在上皮下位置有非组织化的电子致密沉积物。基于PGNMID的诊断,她接受了泼尼松龙治疗,蛋白尿在不到4周的时间内显著减少。尽管PGNMID的临床结局仍有待确定,但MN特征可能是预后良好的一个迹象——这一假设得到了最近报告的支持。肾脏中不存在晚期慢性损伤,如肾小球硬化或肾小管间质纤维化,也可能是本病例预后良好的原因之一。需要对更多的PGNMID病例进行进一步研究,以便将形态学特征和IgG亚类与临床结局相关联,从而证实我们的发现,并进一步巩固这一新疾病实体的临床方面。

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