刚果红阳性的纤维性肾小球肾炎

Congophilic fibrillary glomerulonephritis

青年女性,临床表现为水肿,大量蛋白尿、大量镜下血尿,低蛋白血症,血清肌酐升高,血压偏高。肾活检组织形态学改变为肾小球系膜区及血管袢较多PAS弱阳性、不嗜银的异常物质沉积,免疫荧光肾小球IgG多克隆及κ、λ轻链阳性,电镜下观察到肾小球系膜区大量、基膜内皮下和上皮侧少量直径为14~27 nm纤维丝状物质沉积,刚果红染色肾小球弱阳性,DNAJB9染色肾小球强阳性,最终诊断为刚果红阳性的纤维性肾小球肾炎。

A 22-year-old female presented as massive proteinuria,hematuria,hypoalbuminemia,renal dysfunction and hypertension.Renal biopsies revealed weak PAS-positive and non-argyrophilic deposits in mesangial areas and along GBM.Immunofluerescence indicated glomerular positive staining for polyclonal IgG,κ and λ light chain.Ultrastructral examination indicated randomly arranged fibrils in the mesangium,subendothelial and subepithelial area,14-27 nm in diameter.Congo red staining was weakly positive. DnaJ homolog subfamily B member 9(DNAJB9) staining by Immunohistochemistry showed strong positive. The final diagnosis was congophilic fibrillary glomerulonephritis.