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肾脏病与透析肾移植杂志 ›› 2026, Vol. 35 ›› Issue (4): 307-312.DOI: 10.3969/j.issn.1006⁃298X.2026.04.002

• 论著 • 上一篇    下一篇

足细胞内陷性肾小球病的临床病理分析

  

  • 出版日期:2026-08-28 发布日期:2026-08-31

Clinicopathological analysis of podocyte infolding glomerulopathy cases

  • Online:2026-08-28 Published:2026-08-31

摘要: 目的:分析 52 例足细胞内陷性肾小球病 (PIG) 的临床病理特征,提高对该病的认知,明确电镜的核心诊断价值。 方法:单中心回顾性病例系列研究,纳入 2017 年 1 月至 2024 年 12 月郑州大学第一附属医院肾活检确诊的 52 例 PIG 患者,分析肾活检时的临床病理特征,按是否伴免疫复合物 (IC) 沉积分组,分析电镜下 PIG 分型、病变程度与蛋白尿水平、治疗应答的差异。 结果:PIG 发病年龄集中在 20~59 岁,女性 41 例 (78.85%);29 例 (55.77%) 患者表现为肾病综合征,40 例 (76.92%) 患者合并自身免疫病,其中 37 例 (71.15%) 符合系统性红斑狼疮 (SLE) 诊断,在全部自体肾活检病例中的检出率为 0.11%。40 例 (76.92%) 患者伴 IC 沉积,该组系膜增生、毛细血管内增生及新月体形成率显著高于不伴组 (P<0.05) 但两组在袢坏死肾小球硬化间质小管及血管病变方面无显著差异,且发病年龄、尿蛋白、白蛋白、肌酐亦无差异。电镜下分三种类型:A 型 (足细胞内陷) 3 例 (5.77%),B 型 (微结构 + 内陷) 40 例 (76.92%),C 型 (仅微结构) 9 例 (17.31%)。重度 PIG24 例 (46.15%),B 型和重度 PIG 患者完全缓解率数值更高,但在蛋白尿及治疗应答方面,各型与不同病变程度之间差异无统计学意义。 结论:PIG 发病率低,多合并 SLE 或伴 IC 沉积,电镜是诊断和鉴别诊断的关键手段。

关键词: 足细胞内陷性肾小球病, 临床病理特点, 电镜

Abstract: Objective: Podocyte infolding glomerulopathy (PIG) is a rare glomerular disease, with evidence mainly from case reports and limited large⁃sample studies. This study aims to improve the understanding of this entity and highlight the diagnostic value of electron microscopy (EM). Methods: We conducted a single⁃center retrospective case series of 52 PIG patients diagnosed by renal biopsy at the First Affiliated Hospital of Zhengzhou University between January 2017 and December 2024. Clinicopathological features at biopsy were analyzed, with comparisons between immune complex (IC)⁃positive and IC⁃negative subgroups, and correlations of EM types/severity with proteinuria and treatment response were evaluated. Results: The onset age clustered at 20-59 years, with female predominance (78.85%). Nephrotic syndrome occurred in 55.77%, and 76.92% had autoimmune diseases [71.15% met systemic lupus erythematosus (SLE) criteria]. The detection rate of PIG among all native renal biopsies was 0.11%. IC deposition was present in 76.92%; this group showed more prominent mesangial proliferation, endocapillary proliferation, and crescents (P<0.05), but no differences in loop necrosis, glomerulosclerosis, tubulointerstitial or vascular lesions, nor in age, proteinuria, albumin, or creatinine. Electron microscopy classified PIG into three types: Type A (podocyte infolding) in 5.77%, Type B (microstructures+infolding) in 76.92%, and Type C (microstructures only) in 17.31% of cases; 46.15% were classified as severe. Although Type B and severe PIG demonstrated higher complete remission rates, neither the classification nor the degree of lesion showed statistically significant associations with proteinuria or treatment response. Conclusion: PIG has low incidence, frequently associates with SLE, and often coexists with IC deposition. EM remains the cornerstone for diagnosis and differential diagnosis.