Abstract:

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.

Key words: fibrillary glomerulonephritis, amylodosis, Congo red, fibril