期刊
CEN CASE REPORTS
卷 10, 期 3, 页码 442-447出版社
SPRINGER JAPAN KK
DOI: 10.1007/s13730-021-00585-y
关键词
DnaJ homolog subfamily B member 9 (DNAJB9); Fibrillary glomerulonephritis (FGN); Nephrotic syndrome
A 74-year-old Japanese woman with FGN causing nephrotic syndrome achieved complete remission with steroid therapy, suggesting that some FGN patients can benefit from this treatment approach. Reported cases of DNAJB9-positive FGN among Asians, particularly Japanese individuals, are rare.
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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据