Steroid-sensitive recurrent mesangial proliferative glomerulonephritis with monoclonal IgG deposits
Steroid-sensitive recurrent mesangial proliferative glomerulonephritis with monoclonal IgG deposits
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类固醇敏感性复发性系膜增生性肾小球肾炎伴单克隆 IgG 沉积
DOI：
10.1007/s13730-020-00562-x
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发表时间：
2021
期刊：
影响因子：
1
通讯作者：
Yokoyama Hitoshi
中科院分区：
文献类型：
--
作者：
Nomura Kazutoshi;Miyatake Nobuhiko;Okada Keiichiro;Hayashi Norifumi;Fujimoto Keiji;Adachi Hiroki;Furuichi Kengo;Shimizu Akira;Yokoyama Hitoshi
伴有单克隆免疫球蛋白 G (IgG) 沉积的增殖性肾小球肾炎 (PGNMID) 是一种罕见的肾脏疾病。 PGNMID 的主要病理学发现是肾小球基底膜 (GBM) 上存在单克隆 Ig 沉积。然而，这种肾脏疾病的沉积模式存在一些差异。我们报告一例类固醇敏感性复发性系膜增生型 PGNMID 病例。一名40岁女性发现小腿凹陷性水肿和多尿。大约在第一次就诊前 10 天，根据尿液和血液的实验室数据，她被诊断患有肾病综合征。免疫学和血液学检查未见异常。然而，肾活检标本在光镜下显示轻度系膜细胞增殖和系膜基质积累。关于免疫荧光染色，在 GBM 和系膜区域观察到 IgG、C1q 和 β1c 的颗粒沉积。 GBM 和系膜区域也观察到 IgG3 和 λ 的颗粒状沉积。此外，磷脂酶A2受体抗体和含有血小板反应蛋白1型结构域的7A获得阴性结果。电子显微镜显示高电子密度沉积物主要位于系膜区域。肾活检显示系膜增生性肾小球肾炎，其特征是 IgG3/λ 的单克隆 Ig 沉积。开始类固醇治疗，第36天达到完全缓解。停止类固醇治疗后，蛋白尿复发，第二次肾活检结果与第一次活检几乎相似。然而，通过类固醇治疗实现了完全缓解。这是类固醇敏感性 PGNMID 的罕见复发病例。该病例的病理特征为系膜增生性肾小球肾炎，伴IgG3/λ Ig沉积。
Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits (PGNMID) is a rare kidney disease. The predominant pathological finding of PGNMID is the presence of monoclonal Ig deposits on the glomerular basement membrane (GBM). However, there is some variation in deposition pattern in this kidney disease. We report a case of steroid-sensitive recurrent mesangial proliferative type of PGNMID. A 40-year-old female noticed lower leg pitting edema and polyuria. Approximately 10 days prior to the first clinic visit, she was diagnosed with nephrotic syndrome based on the laboratory data of urine and blood. Immunological and hematological examination revealed no abnormality. However, kidney biopsy specimens showed mild mesangial cell proliferation and mesangial matrix accumulation on light microscopic findings. Regarding immunofluorescence staining, granular deposits of IgG, C1q, and β1c were observed on GBM and mesangial area. Granular deposits of IgG3 andλwere also observed on GBM and mesangial area. Moreover, negative results were obtained for the phospholipase A2 receptor antibody and thrombospondin type-1 domain-containing 7A. Electron microscopy revealed highly electron dense deposits mainly in the mesangial region. Kidney biopsy showed mesangial proliferative glomerulonephritis characterized by monoclonal Ig deposition of IgG3/λ. Steroid therapy was initiated, and complete remission was achieved on day 36. After the discontinuation of steroid therapy, proteinuria recurred and second kidney biopsy findings were almost similar to the first biopsy. However, complete remission was achieved with steroid therapy. This is a rare recurrent case of steroid-sensitive PGNMID. The pathological feature of this case was mesangial proliferative glomerulonephritis with Ig deposition of IgG3/λ.
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影响因子：
4.6
作者：
Omokawa A;Komatsuda A;Hirokawa M;Wakui H
通讯作者：
Wakui H
肾移植后早期复发伴单克隆 IgG 沉积的增殖性肾小球肾炎肾脏病理结果的时间变化
影响因子：
1
作者：
Takayuki Katsuno;Masashi Kato;T. Fujita;N. Tsuboi;R. Hattori;Yasuhiko Ito;S. Maruyama
通讯作者：
S. Maruyama
影响因子：
0.7
作者：
Torrealba J;Gattineni J;Hendricks AR
通讯作者：
Hendricks AR
影响因子：
4.6
作者：
Merhi B;Patel N;Bayliss G;Henriksen KJ;Gohh R
通讯作者：
Gohh R
影响因子：
19.6
作者：
Said, Samar M.;Cosio, Fernando G.;Nasr, Samih H.
通讯作者：
Nasr, Samih H.