A Case of Idiopathic Fibrillary Glomerulonephritis / 대한신장학회잡지

ABSTRACT

A 17-year-old girl was admitted to our hospital due to mild generalized edema. Laboratory tests revealed a serum creatinine was 0.7mg/dL, protein/albumin 6.7/3.5g/dL, cholesterol 190mg/dL, hemoglobin 10.0g/dL, and 24 hour urinary protein 4,40mg/day. Chest X-ray and renal ultrasound were normal. There were no clinical or serologic evidences of paraproteinemia, cryoglobulinemia, light chain disease or systemic lupus erythematosus. Renal biopsy showed membranoproliferative glomerulonephritis-like pattern with lobular accentuation, hypercellularity and diffuse GBM thickening by light microscope. Congo red staining was negative. Granular IgG and C3 deposits were found along the glomerular capillary wall and mesangium by immunofluorescence microscope. Ultrastructurally, abundant subendothelial and mesangial fibrillary deposits were found associated with thickening and wrinkling of GBM. These fibrils, measured about 20-30nm in diameter, were nonbranching and randomly arranged without either periodicity or an organized structure. These findings were compatible with those of fibrillary glomerulonephritis. Thus we report a case of idiopathic fibrillary glomerulonephritis, which is a rare cause of nephrotic syndrome.