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

作　　者：王刘伟[1] 于露[1] 翟子涵 郭艳红 袁亚培 唐琳[1] WANG Liuwei;YU Lu;ZHAI Zihan;GUO Yanhong;YUAN Yapei;TANG Lin(The third Department of Nephrology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院肾脏内三科,郑州450052

出　　处：《肾脏病与透析肾移植杂志》2024年第1期92-96,共5页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：国家自然科学基金(82370727)。

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。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 

分 类 号：R692[医药卫生—泌尿科学]