一例通过肾活检确诊为非狼疮性全套肾病的病例,但在第二次活检时发现了 IgA 肾病。

IF 1 Q4 UROLOGY & NEPHROLOGY
CEN Case Reports Pub Date : 2024-10-01 Epub Date: 2024-02-29 DOI:10.1007/s13730-024-00860-8
Yoichi Iwafuchi, Tetsuo Morioka, Yuko Oyama, Ichiei Narita
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引用次数: 0

摘要

我们描述了一例没有任何基础疾病(包括系统性红斑狼疮)的全房性肾病。一名 40 岁女性因轻度蛋白尿和镜下血尿转诊至我院。根据全室免疫荧光染色的肾脏组织病理学结果,该患者被诊断为免疫复合物介导的肾小球肾炎,以间质增生型为主。除肾脏疾病外,她没有系统性红斑狼疮的临床诊断标准,在整个临床过程中,抗核抗体检测均为阴性。然而,在第二次肾活检中,免疫荧光研究没有检测到 C1q 或 C4,这表明存在免疫球蛋白 A 肾病样模式。患者对皮质类固醇治疗反应良好。我们发现了杂合子 CFHR3-CFHR1 缺失。全房性肾病与 CFHR3-CFHR1 基因缺失之间的关系尚不清楚,但怀疑它对我们病例的组织学模式有影响。这表明非狼疮全房性肾病的发病机制具有多样性,值得进一步研究。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A case of non-lupus full-house nephropathy diagnosed by kidney biopsy but observed IgA nephropathy on second biopsy.

We describe a case of full-house nephropathy without any underlying disease, including systemic lupus erythematosus. A 40-year-old woman was referred to our hospital with mild proteinuria and microscopic hematuria. The patient was diagnosed with immune complex-mediated glomerulonephritis with a predominant mesangioproliferative pattern based on renal histopathological results using full-house immunofluorescence staining. She showed no clinical criteria for the diagnosis of systemic lupus erythematosus, except for kidney disorders, and tested negative for antinuclear antibodies throughout her clinical course. However, in the second kidney biopsy, no C1q or C4 were detected in the immunofluorescence study, suggesting an immunoglobulin A nephropathy-like pattern. The patient responded favorably to corticosteroid treatment. We found a heterozygous CFHR3-CFHR1 deletion. The association between full-house nephropathy and CFHR3-CFHR1 deletion is unknown, but its influence on the histological pattern in our case is suspected. This indicates the diversity in the pathogenesis of non-lupus full-house nephropathy and warrants further investigation.

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来源期刊
CEN Case Reports
CEN Case Reports UROLOGY & NEPHROLOGY-
CiteScore
1.90
自引率
0.00%
发文量
80
期刊介绍: Clinical and Experimental Nephrology (CEN) Case Reports is a peer-reviewed online-only journal, officially published biannually by the Japanese Society of Nephrology (JSN).  The journal publishes original case reports in nephrology and related areas.  The purpose of CEN Case Reports is to provide clinicians and researchers with a forum in which to disseminate their personal experience to a wide readership and to review interesting cases encountered by colleagues all over the world, from whom contributions are welcomed.
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