Suppr超能文献

由多克隆 IgG 演变而来的单克隆 IgG 沉积的膜增生性肾小球肾炎:连续两次肾活检的病例报告。

Membranoproliferative glomerulonephritis with deposition of monoclonal IgG evolved from polyclonal IgG: a case report with two consecutive renal biopsies.

机构信息

Renal Division, Department of Medicine, Peking University First Hospital, Beijing, 100034, People's Republic of China.

Institute of Nephrology, Peking University, Beijing, 100034, People's Republic of China.

出版信息

BMC Nephrol. 2019 Jul 22;20(1):275. doi: 10.1186/s12882-019-1453-4.

Abstract

BACKGROUND

Proliferative glomerulonephritis with monoclonal Immunoglobulin (G) deposits (PGNMID) is a rare kind of MGRS with intact monoclonal IgG deposition. Seventy percent of PGNMID patients were negative for M-spike.

CASE PRESENTATION

A 51-year-old Chinese woman presented with 16-month history of chronic nephritic syndrome. Her first biopsy showed a MPGN pattern, and the IF showed polyclonal IgG deposition but with IgG3λ dominance, MGRS was highly suspected. But the serum/urine IFE and bone marrow examination was negative for monoclonal gammopathy. She was treated with RAS inhibitors, and monitored carefully in the outpatient clinic. When the proteinuria was not controlled by RAS inhibitors, immunosuppressive agents were initiated. The second biopsy was done due to her acute kidney injury 9 months later, showing a MPGN pattern with acute tubulointerstitial disease, but the IF showed monoclonal IgG3λ deposition. The κ light chain, IgG1, IgG2 and IgG4 were absent. Electron microscopic examination revealed electron-dense deposits in the mesangial, subendothelial and subepithelial area which is the same as the first renal biopsy. The final diagnose of this patient was PGNMID (IgG3λ) with non-organized deposits. Repeated serum/urine IFE and free light chain still failed to identify monoclonal gammopathy. The patient was treated with steroid and cyclophosphamide, and her serum creatinine decreased.

CONCLUSIONS

Some of the PGNMID patients may be derived from polyclonal immune complex mediated glomerulonephritis.

摘要

背景

伴有单克隆免疫球蛋白(G)沉积的增殖性肾小球肾炎(PGNMID)是一种罕见的 MGRS,其具有完整的单克隆 IgG 沉积。70%的 PGNMID 患者 M 峰为阴性。

病例介绍

一名 51 岁的中国女性,有 16 个月的慢性肾炎综合征病史。她的首次肾活检显示为 MPGN 模式,免疫荧光检查显示多克隆 IgG 沉积,但 IgG3λ 占优势,高度怀疑为 MGRS。然而,血清/尿液免疫电泳和骨髓检查均未发现单克隆丙种球蛋白病。她接受了 RAS 抑制剂治疗,并在门诊进行了密切监测。当 RAS 抑制剂不能控制蛋白尿时,开始使用免疫抑制剂。9 个月后,由于急性肾损伤,进行了第二次肾活检,结果显示 MPGN 模式伴急性肾小管间质性疾病,但免疫荧光检查显示单克隆 IgG3λ 沉积。κ 轻链、IgG1、IgG2 和 IgG4 均缺失。电子显微镜检查显示电子致密物沉积在系膜、内皮下和上皮下区域,与首次肾活检相同。最终诊断为 PGNMID(IgG3λ)伴非组织化沉积。反复进行血清/尿液免疫电泳和游离轻链检查仍未能识别单克隆丙种球蛋白病。该患者接受了类固醇和环磷酰胺治疗,其血清肌酐降低。

结论

一些 PGNMID 患者可能来源于多克隆免疫复合物介导的肾小球肾炎。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/acc8/6647130/352d6b704a81/12882_2019_1453_Fig1_HTML.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验