增殖性肾小球肾炎伴单克隆 IgG 沉积：一例独特病例，病程超过 46 年。

Proliferative glomerulonephritis with monoclonal IgG deposits: a unique case with a clinical course of over 46 years.

机构信息

School of Medicine, University of Texas Medical Branch, Galveston, TX, USA.

Department of Pathology, University of Texas Medical Branch, Galveston, TX, USA.

出版信息

BMC Nephrol. 2023 Apr 25;24(1):109. doi: 10.1186/s12882-023-03178-2.

DOI:10.1186/s12882-023-03178-2

PMID:37098508

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10126991/

Abstract

BACKGROUND

Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a rare entity first described in 2004. We present a case of PGNMID with recurrent hematuria and nephrotic range proteinuria with three biopsies over 46 years.

CASE PRESENTATION

A 79-year-old Caucasian female presents with a history of two separate episodes of biopsy-proven recurrent GN over a course of 46 years. Both biopsies from 1974, and 1987 were reported as membranoproliferative GN (MPGN). The patient presented in 2016 for the third time with symptoms of fluid overload, slight worsening in renal function, and proteinuria along with glomerular hematuria. A third kidney biopsy was performed, and the final diagnosis was proliferative glomerulonephritis with monoclonal IgG/κ deposits.

CONCLUSION

With three renal biopsies obtained over 46 years, our case opens a unique window into the natural history of PGNMID. The three biopsies demonstrate the immunologic and morphologic evolution of PGNMID in the kidney.

摘要

背景

伴单克隆 IgG 沉积的增殖性肾小球肾炎（PGNMID）于 2004 年首次被描述，是一种罕见疾病。我们报告了一例 PGNMID 患者，其表现为血尿和肾病范围蛋白尿，在 46 年内进行了三次肾活检。

病例介绍

一名 79 岁白人女性因两次单独的经活检证实的复发性肾小球肾炎病史就诊，病程长达 46 年。1974 年和 1987 年的两次活检均报告为膜增殖性肾小球肾炎（MPGN）。患者于 2016 年因第三次出现液体超负荷、肾功能轻度恶化以及伴有肾小球血尿的蛋白尿而就诊。进行了第三次肾活检，最终诊断为伴单克隆 IgG/κ 沉积的增殖性肾小球肾炎。