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伴有单克隆IgG4沉积的膜性肾病及相关IgG4相关性肺病。

Membranous nephropathy with monoclonal IgG4 deposits and associated IgG4-related lung disease.

作者信息

Omokawa Ayumi, Komatsuda Atsushi, Hirokawa Makoto, Wakui Hideki

机构信息

Department of General Internal Medicine and Clinical Laboratory Medicine , Akita University Graduate School of Medicine , Akita , Japan.

Department of Hematology, Nephrology and Rheumatology , Akita University Graduate School of Medicine , Akita , Japan.

出版信息

Clin Kidney J. 2014 Oct;7(5):475-8. doi: 10.1093/ckj/sfu077. Epub 2014 Jul 25.

DOI:10.1093/ckj/sfu077
PMID:25878779
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4379336/
Abstract

A 62-year-old woman was admitted for nephrotic syndrome and lung tumor. A renal biopsy showed membranous features of the glomeruli. Immunofluorescence studies revealed granular IgG4-κ deposits along with the glomerular basement membrane. Electron microscopy revealed granular electron-dense deposits. Further study denied multiple myeloma. Light microscopy of the resected lung tumor revealed IgG4-related lung disease with no malignancy. Steroid therapy induced a remission of the nephrotic syndrome, with no recurrence of the lung tumor. We consider that this is the first case of a proliferative glomerulonephritis with monoclonal IgG deposits of IgG4 subclass, and a rare concurrence with IgG4-related disease.

摘要

一名62岁女性因肾病综合征和肺部肿瘤入院。肾活检显示肾小球具有膜性特征。免疫荧光研究显示沿肾小球基底膜有颗粒状IgG4-κ沉积。电子显微镜检查发现颗粒状电子致密沉积物。进一步检查排除了多发性骨髓瘤。切除的肺部肿瘤的光学显微镜检查显示为IgG4相关肺部疾病，无恶性肿瘤。类固醇治疗使肾病综合征缓解，肺部肿瘤未复发。我们认为这是首例具有IgG4亚类单克隆IgG沉积的增殖性肾小球肾炎病例，且与IgG4相关疾病罕见并发。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/8e84912edd4f/sfu07705.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/766b568d3574/sfu07701.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/283bdd9cbc50/sfu07702.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/4cf05cfe08b8/sfu07703.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/7818aed1655b/sfu07704.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/8e84912edd4f/sfu07705.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/766b568d3574/sfu07701.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/283bdd9cbc50/sfu07702.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/4cf05cfe08b8/sfu07703.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/7818aed1655b/sfu07704.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f69f/4379336/8e84912edd4f/sfu07705.jpg

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Clin Kidney J. 2013 Oct;6(5):486-490. doi: 10.1093/ckj/sft062. Epub 2013 Aug 13.
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