Fibrillary glomerulonephritis accompany with crescentic glomerulonephritis
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.
rituximabcrescentic glomerulonephritisDNAJB9 related fibrillary glomerulonephritis
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