伪装成膜性肾病的荚膜细胞折叠肾小球病--共同的发病机制?

Pub Date : 2024-07-01 Epub Date: 2024-06-24 DOI:10.25259/ijn_209_23
Smita Mary Matthai, Liam Hems, Yee Wah Tsang, Yogita Aggarwal, Huda Mahmoud, Kishore Gopalakrishnan
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引用次数: 0

摘要

荚膜细胞折叠性肾小球病(PIG)是一种罕见的病理实体,通过电子显微镜显示荚膜细胞向肾小球基底膜弥漫性折叠而确诊。我们报告了英国首例表现出 PIG 典型超微结构特征的病例,患者为一名男性,患有 II 型糖尿病、高血压和常见变异性免疫缺陷。肾活检发现磷脂酶 A2 受体(PLA2R)免疫印迹阳性的膜性肾病(MN),但血清中没有 PLA2R 抗体。电子显微镜检查发现,荚膜细胞弥漫性折叠进入肾小球基底膜,基底膜内弥漫性分布着病理特征性的微球状和微管状团块,可诊断为 PIG。我们推测 PIG 和 MN 之间存在共同的病理机制联系,并强调了这两种疾病的重叠特征。
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Podocyte Infolding Glomerulopathy Masquerading as Membranous Nephropathy - A Shared Pathogenesis?

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.

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