Abstract: ＴThe 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.

Key words: rituximab, crescentic glomerulonephritis, DNAJB9 related fibrillary glomerulonephritis