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足细胞内褶肾小球病合并磷脂酶 A2 受体阳性膜性肾病一例并文献复习

Podocytic Infolding Glomerulopathy in a Patient with Phospholipase A2 Receptor-Positive Membranous Nephropathy and Review of the Literature.

机构信息

Renal Division, University of Massachusetts Medical Center, Worcester, Massachusetts, USA.

Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts, USA.

出版信息

Nephron. 2021;145(5):496-502. doi: 10.1159/000515769. Epub 2021 May 7.

Abstract

Podocytic infolding glomerulopathy (PIG) is a rare diagnosis based on characteristic histopathologic findings on renal biopsy and was proposed as a new entity about a decade ago. It is a type of podocytic injury, characterized by invagination of the podocyte cell membrane (cytoplasmic projections from podocytes) into the glomerular basement membrane. The presence of microspheres and/or microtubules on electron microscopy is the characteristic finding. PIG is most often associated with autoimmune conditions like systemic lupus erythematosus. We report pathologic findings typical of PIG in a patient with phospholipase A2 receptor antibody-positive membranous nephropathy. She was treated with rituximab and responded well with decrease in proteinuria to the sub-nephrotic range.

摘要

足细胞内褶肾小球病(PIG)是一种罕见的诊断,基于肾活检的特征性组织病理学发现,大约十年前被提出作为一种新的实体。它是一种足细胞损伤的类型,特征是足细胞细胞膜(来自足细胞的细胞质突起)向内褶入肾小球基底膜。电子显微镜下存在微球体和/或微管是其特征性发现。PIG 最常与自身免疫性疾病如系统性红斑狼疮有关。我们报告了一名磷脂酶 A2 受体抗体阳性的膜性肾病患者的典型 PIG 病理发现。她接受了利妥昔单抗治疗,蛋白尿减少到接近肾病范围,病情得到了很好的缓解。

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