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磷脂酶A2受体(PLA2R)阳性膜性肾病中系膜电子致密沉积物的患病率

The Prevalence of Mesangial Electron-Dense Deposits in PLA2R-Positive Membranous Nephropathy.

作者信息

Giannini Gabriel, Arend Lois J

机构信息

Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

出版信息

Nephron. 2022;146(2):167-171. doi: 10.1159/000519912. Epub 2021 Nov 19.

Abstract

INTRODUCTION

Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults and can be primary or secondary. The antigenic target of antibodies in 70% of primary cases is phospholipase A2 receptor (PLA2R). The presence or absence of mesangial electron-dense deposits has been used to distinguish between primary and secondary MN. Mesangial deposits suggest MN due to lupus, infection, or other causes, though they are reported to occur in approximately 10% of primary MN. Staining for PLA2R is now frequently used for confirming a diagnosis of primary MN. If mesangial deposits predict a secondary cause, they should be more frequent in PLA2R-negative biopsies.

METHODS

A review of institutional kidney biopsies between March 2017 and June 2020 identified all cases of MN. Cases with a diagnosis of lupus or near "full-house" staining by immunofluorescence microscopy (IF) were excluded. Light microscopy, IF, and electron microscopy (EM) were performed. PLA2R staining was performed by IF. EM for all cases was reviewed and electron-dense deposit location, distribution, and size were determined.

RESULTS

Ninety-three cases of MN were identified, of which 86 had both PLA2R staining and EM performed. Of these, 51 cases (59%) were positive for PLA2R and 35 (41%) were negative. Mesangial electron-dense deposits were present in 22 (25.6%) of the 86 cases, including 27.5% (14/51) of PLA2R-positive cases and 22.8% (8/35) of PLA2R-negative cases. No difference was seen in size or distribution of deposits, or other features considered suggestive of secondary MN.

CONCLUSION

PLA2R-negative cases were not more likely to have mesangial deposits than PLA2R-positive cases. Mesangial deposits should not be used as an indicator of secondary MN.

摘要

引言

膜性肾病(MN)是成人肾病综合征的常见病因,可分为原发性或继发性。70%的原发性病例中抗体的抗原靶点是磷脂酶A2受体(PLA2R)。系膜电子致密沉积物的有无已被用于区分原发性和继发性MN。系膜沉积物提示MN由狼疮、感染或其他原因引起,尽管据报道约10%的原发性MN中会出现这种情况。目前,PLA2R染色常用于确诊原发性MN。如果系膜沉积物预示继发性病因,那么它们在PLA2R阴性活检中应更常见。

方法

回顾2017年3月至2020年6月间机构肾脏活检病例,确定所有MN病例。排除诊断为狼疮或免疫荧光显微镜(IF)显示近乎“满堂亮”染色的病例。进行了光镜、IF和电子显微镜(EM)检查。通过IF进行PLA2R染色。对所有病例的EM进行复查,确定电子致密沉积物的位置、分布和大小。

结果

共确定93例MN病例,其中86例同时进行了PLA2R染色和EM检查。其中,51例(59%)PLA2R阳性,35例(4例)PLA2R阴性。86例中有22例(25.6%)存在系膜电子致密沉积物,包括51例PLA2R阳性病例中的27.5%(14/51)和35例PLA2R阴性病例中的22.8%(8/35)。在沉积物的大小、分布或其他提示继发性MN的特征方面未发现差异。

结论

PLA2R阴性病例与PLA2R阳性病例相比,系膜沉积物的可能性并无增加。系膜沉积物不应作为继发性MN的指标。

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