表现为新月体性肾小球肾炎的纤维性肾小球病
Fibrillary glomerulonephritis accompany with crescentic glomerulonephritis
王刘伟 1于露 1翟子涵 1郭艳红 1袁亚培 1唐琳1
作者信息
- 1. 郑州大学第一附属医院肾脏内三科(郑州,450052)
- 折叠
摘要
34 岁女性患者,临床表现为水肿、高血压、大量蛋白尿、镜下血尿、低蛋白血症、血清肌酐升高.肾活检病理光镜示大量新月体形成伴袢坏死,电镜示上皮下、基膜内及系膜区杂乱分布的纤维样结构(直径 12～30 nm)沉积,肾小球免疫组化染色DNAJ热休克蛋白家族成员B9(DNAJB9)阳性,诊断为DNAJB9 相关纤维性肾小球病、新月体性肾小球肾炎.给予利妥昔单抗、吗替麦考酚酯、糖皮质激素等治疗,获得了较好的临床疗效.
Abstract
The clinical manifestations of a 34-year-old female we reported were edema,hypertension,massive proteinuria,microscopic hematuria,hypoalbuminemia and elevated creatinine.Renal biopsy revealed the formation of a large number of crescents with necrosis under the light microscope,and the deposition of fibrous structures with a diameter of 12-30nm in the subepithelial,basement membrane,and mesangial areas under the electron microscope.Immunohistochemical staining of the glomerulus showed positive staining for DNAJ heat shock protein family member B9(DNAJB9),indicating a diagnosis of DNAJB9 related fibrillary glomerulonephritis(FGN).The treatment with rituximab,mycophenolate mofetil,and glucocorticoids has achieved good clinical efficacy.
关键词
利妥昔单抗/新月体性肾小球肾炎/DNAJ热休克蛋白家族成员B9相关纤维性肾小球病Key words
rituximab/crescentic glomerulonephritis/DNAJB9 related fibrillary glomerulonephritis引用本文复制引用
出版年
2024
NETL
NSTL
万方数据