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点状足细胞IgG染色无法区分原发性与继发性微小病变病。

Punctate Podocyte IgG Staining Does Not Differentiate Primary from Secondary Minimal Change Disease.

作者信息

Leong Matthew, Lee Chen Yu Jamie, Yamashita Michifumi, Kiyozawa Daisuke, Han Man-Hoon, Nast Cynthia C

机构信息

Department of Pathology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.

Department of Anatomic Pathology, Kyushu University, Fukuoka, Japan.

出版信息

Glomerular Dis. 2025 Jun 30;5(1):316-327. doi: 10.1159/000547193. eCollection 2025 Jan-Dec.

Abstract

INTRODUCTION

Punctate IgG staining of podocytes ("dusting") identified by immunofluorescence recently has been described in a subset of minimal change disease (MCD), possibly correlating with anti-nephrin or other autoantibodies. Whether dusting is associated with other clinicopathologic features of MCD remains unclear, but identification of these associations could provide insight into MCD mechanisms, prognosis, and therapeutic strategies.

METHODS

Cases with a diagnosis of MCD over 8.5 years at one institution were retrospectively reviewed, including reexamination of IgG immunofluorescence and electron microscopy when necessary. Demographic, clinical, and ultrastructural feature data were collected. Cases were divided into presumed primary and secondary MCD based on clinical associations and they were assessed for dusting frequency.

RESULTS

A total of 371 cases were included, of which 73% were primary MCD and 16.4% were pediatric. Dusting frequency among MCD etiologies ranged from 45 to 63% and did not differ between children and adults, or in primary versus any secondary MCD association. Dusting was positively correlated with the degree of podocyte foot process effacement ( < 0.005) and actin cytoskeletal condensation ( = 0.001). Otherwise, dusting was not associated with other ultrastructural features of proteinuric kidney disease or with podocyte ballooning clusters.

CONCLUSION

Podocyte IgG dusting was not specific for any one etiologic trigger of MCD or age category. This suggests that autoantibodies may induce MCD irrespective of a primary (idiopathic) versus a presumed secondary cause, which may alter the diagnostic and therapeutic approach. Additionally, podocyte ultrastructural features associated with dusting may reflect mechanisms of autoantibody-induced injury, or may represent a feature of disease severity and/or temporal progression.

摘要

引言

免疫荧光法鉴定的足细胞点状IgG染色(“尘染”)最近在一小部分微小病变性肾病(MCD)中被描述,可能与抗nephrin或其他自身抗体相关。“尘染”是否与MCD的其他临床病理特征相关尚不清楚,但确定这些关联可能有助于深入了解MCD的发病机制、预后及治疗策略。

方法

回顾性分析某机构8.5年期间诊断为MCD的病例,必要时重新检查IgG免疫荧光和电子显微镜。收集人口统计学、临床和超微结构特征数据。根据临床关联将病例分为原发性和继发性MCD,并评估“尘染”频率。

结果

共纳入371例病例,其中73%为原发性MCD,16.4%为儿童病例。MCD各病因中的“尘染”频率在45%至63%之间,儿童和成人之间、原发性与任何继发性MCD关联之间均无差异。“尘染”与足细胞足突消失程度呈正相关(<0.005),与肌动蛋白细胞骨架凝聚呈正相关(=0.001)。此外,“尘染”与蛋白尿性肾病的其他超微结构特征或足细胞气球样聚集无关。

结论

足细胞IgG“尘染”并非MCD任何一种病因或年龄组所特有。这表明自身抗体可能诱发MCD,而与原发性(特发性)或推测的继发性病因无关,这可能会改变诊断和治疗方法。此外,与“尘染”相关的足细胞超微结构特征可能反映自身抗体诱导损伤的机制,或可能代表疾病严重程度和/或时间进展的特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6e0f/12310193/a76aa8baf569/gdz-2025-0005-0001-547193_F01.jpg

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