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一例以膜性特征为主伴单克隆IgG沉积的增生性肾小球肾炎

A Case of Proliferative Glomerulonephritis with Monoclonal IgG Deposits That Showed Predominantly Membranous Features.

作者信息

Shimohata Homare, Ohgi Kentaro, Maruyama Hiroshi, Miyamoto Yasunori, Takayashu Mamiko, Hirayama Kouichi, Kobayashi Masaki

机构信息

Department of Nephrology, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan.

出版信息

Case Rep Nephrol. 2017;2017:1027376. doi: 10.1155/2017/1027376. Epub 2017 Oct 25.

Abstract

In 2004, the novel category of monoclonal IgG deposition disease has been proposed and termed "proliferative glomerulonephritis with monoclonal IgG deposits" (PGNMID). This disease is characterized by membranoproliferative glomerulonephritis and staining for a single light-chain isotype and gamma heavy-chain subclass. A 76-year-old male who had monoclonal gammopathy was referred to our hospital because of proteinuria. The renal biopsy showed diffuse thickening of the glomerular capillary walls with focal mesangial proliferation. On immunofluorescence study, only IgG1 among the four subclasses and lambda light chains were detected mainly in the glomerular capillary walls. From these results, we diagnosed our case as PGNMID showing predominantly membranous features. Almost all pathological findings on light microscopy of PGNMID are membranoproliferative GN or endocapillary proliferative GN, while membranous GN cases are rare. Here, we present the case of PGNMID that showed predominantly membranous features on light microscopy.

摘要

2004年,一种新型的单克隆IgG沉积病被提出,并命名为“伴单克隆IgG沉积的增生性肾小球肾炎”(PGNMID)。这种疾病的特征是膜增生性肾小球肾炎以及对单一轻链同种型和γ重链亚类的染色。一名患有单克隆丙种球蛋白病的76岁男性因蛋白尿转诊至我院。肾活检显示肾小球毛细血管壁弥漫性增厚,伴有局灶性系膜增生。免疫荧光研究显示,在四个亚类中仅检测到IgG1,且λ轻链主要存在于肾小球毛细血管壁。根据这些结果,我们将该病例诊断为主要表现为膜性特征的PGNMID。PGNMID光镜下几乎所有病理表现为膜增生性肾小球肾炎或毛细血管内增生性肾小球肾炎,而膜性肾小球肾炎病例罕见。在此,我们报告一例光镜下主要表现为膜性特征的PGNMID病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1022/5676379/492dea26510b/CRIN2017-1027376.001.jpg

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