Concurrent Crystalline Light-Chain Proximal Tubulopathy and Membranous Nephropathy: A Case Report and Literature Review

IF 3.2 Q1 UROLOGY & NEPHROLOGY

Kidney Medicine Pub Date : 2024-05-01 DOI:10.1016/j.xkme.2024.100816

Huizi Zhang, Chunyun Zhang, Hua Su

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Abstract

Light-chain proximal tubulopathy (LCPT) is typically characterized by the intracytoplasmic deposition of light chains within the proximal tubular epithelial cells, which is usually classified into crystalline and noncrystalline subgroups. Membranous nephropathy (MN) is a common glomerular disease characterized by diffused subepithelial electron-dense deposits along the capillary loop accompanied by the effacement and microvillus transformation of the foot process. Here, we report a biopsy-confirmed case of a concurrence of LCPT with crystals (κ light chains restricted) and antigen-undetermined MN in a male patient. The patient presented with low-molecular-weight proteinuria, increased serum creatinine levels, and incomplete Fanconi syndrome. To our knowledge, this is the first report of a concurrence of LCPT and independent MN of unknown target antigens, which may enrich our recognition of monoclonal gammopathy of renal significance with synchronous MN.

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并发结晶轻链近端肾小管病变和膜性肾病：病例报告和文献综述

轻链近端肾小管病（LCPT）的典型特征是近端肾小管上皮细胞内轻链的胞浆内沉积，通常分为结晶亚组和非结晶亚组。膜性肾病（MN）是一种常见的肾小球疾病，其特征是沿毛细血管襻的上皮下弥漫性电子致密沉积，同时伴有足突的脱落和微绒毛变。在此，我们报告了一例经活检证实的男性患者同时患有 LCPT 与晶体（κ轻链受限）和抗原未确定的 MN 的病例。患者表现为低分子量蛋白尿、血清肌酐水平升高和不完全范可尼综合征。据我们所知，这是第一例报告 LCPT 和目标抗原不明的独立 MN 同时存在的病例，这可能会丰富我们对肾脏单克隆丙种球蛋白病与同步 MN 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

Kidney Medicine Medicine-Internal Medicine

CiteScore

4.80

自引率

5.10%

发文量

176

审稿时长

12 weeks