Complete remission of DnaJ homolog subfamily B member 9-positive fibrillary glomerulonephritis following steroid monotherapy in an elderly Japanese woman
Complete remission of DnaJ homolog subfamily B member 9-positive fibrillary glomerulonephritis following steroid monotherapy in an elderly Japanese woman
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一位日本老年妇女在接受类固醇单药治疗后，DnaJ 同源物 B 亚家族成员 9 阳性纤维性肾小球肾炎完全缓解
DOI：
10.1007/s13730-021-00585-y
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发表时间：
2021
期刊：
影响因子：
1
通讯作者：
T. Oda
中科院分区：
文献类型：
--
作者：
Takahiro Uchida;Shuuhei Komatsu;Takashi Sakai;A. Kojima;S. Iwama;K. Sugisaki;T. Oda
一位74岁的日本女性因全身水肿及大量蛋白尿而转介至我科。她被临床诊断为肾病综合征，肾活检显示膜增生性肾小球肾炎，伴有明显的巨噬细胞肾小球浸润和全室免疫荧光肾小球沉积。此外，通过电子显微镜发现直径约12 nm的随机排列的无分支纤维，并且DnaJ同源物亚家族B成员9（DNAJB 9）（最近鉴定的肾小球肾炎（FGN）的诊断生物标志物）的免疫染色显示阳性结果，从而确认FGN的诊断。开始类固醇治疗后，她的肾病综合征完全缓解，并一直维持下去。FGN是一种不常见的肾小球疾病，亚洲人（尤其是日本人群）中报告的DNAJB 9阳性FGN病例很少见。目前还没有确定的治疗方案，其肾脏预后通常不利。目前的情况表明，一些FGN患者可以通过类固醇单药治疗获得良好的临床结果。
A 74-year-old Japanese woman was referred to our department because of anasarca and massive proteinuria. She was clinically diagnosed with nephrotic syndrome, and renal biopsy showed membranoproliferative glomerulonephritis accompanied by marked glomerular infiltration with macrophages and full-house immunofluorescence glomerular deposition. Furthermore, randomly arranged nonbranching fibrils, approximately 12 nm in diameter, were found by electron microscopy, and immunostaining for DnaJ homolog subfamily B member 9 (DNAJB9), a recently identified diagnostic biomarker of fibrillary glomerulonephritis (FGN), showed positive result, thereby confirming the diagnosis of FGN. Steroid treatment was initiated, and she obtained complete remission of nephrotic syndrome and has maintained it. FGN is an uncommon form of glomerular disease, and reported cases of DNAJB9-positive FGN among Asians, particularly among Japanese population, are rare. There have been no established therapeutic regimens and its renal prognosis is generally unfavorable. The present case suggests that some patients with FGN can achieve favorable clinical outcomes through steroid monotherapy.
DOI：
10.2215/cjn.03870319
发表时间：
2019-12-06
影响因子：
9.8
作者：
Andeen, Nicole K.;Troxell, Megan L.;Smith, Kelly D.
通讯作者：
Smith, Kelly D.
影响因子：
13.6
作者：
Andeen, Nicole K.;Yang, Han-Yin;Smith, Kelly D.
通讯作者：
Smith, Kelly D.