IgG4-related disease complicated by PLA2R-associated membranous nephropathy: A case report

IF 16.4 1区化学 Q1 CHEMISTRY, MULTIDISCIPLINARY

Accounts of Chemical Research Pub Date : 2024-07-25 DOI:10.1515/biol-2022-0921

Meichun Huang, Jun Liu, Xiuxiu Li

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Abstract

IgG4-related tubulointerstitial nephritis (IgG4-related TIN) is the prevalent clinical manifestation of IgG4-related diseases (IgG4-RD). However, there are limited cases of IgG4-RD occurring with membranous nephropathy (MN) in the absence of phospholipase A2 receptor (PLA2R). There have been no indications of treatment using Tripterygium wilfordii. This study reported a rare case of IgG4-RD with PLA2R-associated MN without any of the distinct IgG4-related TIN. The patient was treated effectively with T. wilfordii. A 71-year-old patient was admitted to the medical facility after presenting with a 1 month history of edema and 8 months of albuminuria. The renal biopsy tissue examination confirmed the presence of MN (phase II) in the absence of pathological manifestations of IgG4-related TIN. Immunohistochemistry identified PLA2R++ (granular capillaries). The serum PLA2R antibody titer was 1:180 (1:20). The patient met the diagnosis with IgG4-RD. Over 8 years of follow-up, the patient was effectively treated with low-dose hormones and T. wilfordii, without any adverse effects. This MN is considered a unique form of IgG4-RD, regardless of whether PLA2R antibodies are present or not. Research suggests that T. wilfordii could be a promising option for elderly people with IgG4-related MN, as it has been found to have fewer adverse effects.

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IgG4相关疾病并发PLA2R相关膜性肾病：病例报告

IgG4相关性肾小管间质性肾炎（IgG4-related TIN）是IgG4相关性疾病（IgG4-RD）的主要临床表现。然而，在没有磷脂酶 A2 受体（PLA2R）的情况下，IgG4-RD 并发膜性肾病（MN）的病例并不多。目前还没有使用威灵仙治疗的迹象。本研究报告了一例罕见的IgG4-RD伴有PLA2R相关的MN，但没有任何明显的IgG4相关TIN。患者接受了T. wilfordii的有效治疗。一名 71 岁的患者因 1 个月的水肿史和 8 个月的白蛋白尿史入院。肾活检组织检查证实存在 MN（II 期），但没有 IgG4 相关 TIN 的病理表现。免疫组化结果显示 PLA2R++（颗粒状毛细血管）。血清 PLA2R 抗体滴度为 1:180（1:20）。患者符合 IgG4-RD 诊断。在 8 年的随访中，患者接受了低剂量激素和 T. wilfordii 的有效治疗，未出现任何不良反应。无论是否存在 PLA2R 抗体，这种 MN 都被认为是 IgG4-RD 的一种独特形式。研究表明，对于患有IgG4相关性MN的老年人来说，威灵仙可能是一种很有前景的选择，因为它的不良反应较少。

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

Accounts of Chemical Research 化学-化学综合

CiteScore

31.40

自引率

1.10%

发文量

312

审稿时长

2 months

期刊介绍： Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance. Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.