EXT1/EXT2-Associated Membranous Nephropathy Secondary to TAFRO Syndrome: A Case Report.

IF 1.8 4区医学 Q2 UROLOGY & NEPHROLOGY

Nephron Pub Date : 2025-08-02 DOI:10.1159/000547782

Hisashi Kamido, Yuki Oba, Shigekazu Kurihara, Masayuki Yamanouchi, Tatsuya Suwabe, Kei Kono, Kenichi Ohashi, Shinya Yamamoto, Takahiro Tsuji, Yoshifumi Ubara, Naoki Sawa

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引用次数: 0

Abstract

Introduction: Renal involvement in TAFRO syndrome usually is present as acute kidney injury with oligoproteinuria. Renal pathology is typically characterized by glomerular microangiopathy without immune deposits, and there have been no reports of membranous nephropathy. While idiopathic multicentric Castleman disease (iMCD), which shares a similar pathophysiology with TAFRO syndrome, has documented several cases of membranous nephropathy, the underlying mechanisms remain unclear.

Case presentation: We present a case of TAFRO syndrome presenting with nephrotic syndrome, and kidney biopsy revealed exostosin 1/exostosin 2 (EXT1/EXT2)-associated membranous nephropathy. EXT1/EXT2 is considered a potential target antigen in autoimmune membranous nephropathy, suggesting their potential pathogenic role in this case. In iMCD cases with membranous nephropathy, IL-6 levels tend to be slightly low, while VEGF levels are significantly elevated, as seen in the present case. This cytokine profile may contribute to the differences in renal pathological findings and may also be involved in the response to treatment.

Conclusion: This case may enhance our understanding of the pathophysiology of membranous nephropathy in TAFRO syndrome and iMCD.

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继发于TAFRO综合征的EXT1/ ext2相关膜性肾病1例报告

简介：TAFRO综合征的肾脏受累通常表现为急性肾损伤伴少蛋白尿。肾脏病理的典型特征是肾小球微血管病变，没有免疫沉积，没有膜性肾病的报道。虽然特发性多中心Castleman病（iMCD）与TAFRO综合征具有相似的病理生理机制，但已记录了几例膜性肾病，其潜在机制尚不清楚。病例介绍：我们报告一例TAFRO综合征，表现为肾病综合征，肾活检显示Exostosin 1/Exostosin 2 （EXT1/EXT2）相关的膜性肾病。EXT1/EXT2被认为是自身免疫性膜性肾病的潜在靶抗原，提示它们在本病例中的潜在致病作用。在iMCD合并膜性肾病的病例中，IL-6水平往往略低，而VEGF水平明显升高，如本病例所示。这种细胞因子谱可能导致肾脏病理结果的差异，也可能参与对治疗的反应。结论：本病例有助于加深对TAFRO综合征和iMCD的膜性肾病病理生理的认识。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Nephron UROLOGY & NEPHROLOGY-

CiteScore

5.00

自引率

0.00%

发文量

期刊介绍： ''Nephron'' comprises three sections, which are each under the editorship of internationally recognized leaders and served by specialized Associate Editors. Apart from high-quality original research, ''Nephron'' publishes invited reviews/minireviews on up-to-date topics. Papers undergo an innovative and transparent peer review process encompassing a Presentation Report which assesses and summarizes the presentation of the paper in an unbiased and standardized way.