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伪装成膜性肾病的足细胞内褶肾小球病——共同的发病机制?

Podocyte Infolding Glomerulopathy Masquerading as Membranous Nephropathy - A Shared Pathogenesis?

作者信息

Matthai Smita Mary, Hems Liam, Tsang Yee Wah, Aggarwal Yogita, Mahmoud Huda, Gopalakrishnan Kishore

机构信息

Central EM Facility, Christian Medical College, Vellore, Tamil Nadu, India.

Department of Cellular Pathology, University Hospital Coventry and Warwickshire, Coventry, UK.

出版信息

Indian J Nephrol. 2024 Jul-Aug;34(4):397-400. doi: 10.25259/ijn_209_23. Epub 2024 Jun 24.

Abstract

Podocyte infolding glomerulopathy (PIG) is a rare pathological entity, diagnosed by electron microscopic demonstration of diffuse infolding of the podocytes into the glomerular basement membranes. We report the first case from United Kingdom exhibiting typical ultrastructural features of PIG in a male with Type II diabetes mellitus, hypertension and common variable immune deficiency. Renal biopsy revealed phospholipase A2 receptor (PLA2R) immunostain positive membranous nephropathy (MN) but no serum PLA2R antibodies. Diffuse infolding of the podocytes into the glomerular basement membranes along with pathognomonic microspherular and microtubular intra basement membrane clusters distributed diffusely and globally were noted on electron microscopy, diagnostic of PIG. We postulate a shared pathomechanistic link between PIG and MN, highlighting the overlapping features of both conditions.

摘要

足细胞内褶性肾小球病(PIG)是一种罕见的病理实体,通过电子显微镜显示足细胞向肾小球基底膜的弥漫性内褶来诊断。我们报告了英国首例具有PIG典型超微结构特征的病例,该患者为男性,患有II型糖尿病、高血压和常见变异型免疫缺陷。肾活检显示磷脂酶A2受体(PLA2R)免疫染色阳性的膜性肾病(MN),但血清中无PLA2R抗体。电子显微镜检查发现足细胞向肾小球基底膜弥漫性内褶,同时在基底膜内弥漫性和全身性分布有特征性的微球和微管簇,诊断为PIG。我们推测PIG和MN之间存在共同的发病机制联系,突出了这两种疾病的重叠特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2f4/11326786/25fb98b3b732/IJN-34-4-397-g1.jpg

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