A case of glomerulonephritis showing remarkable segmental extracapillary proliferation; Is this a new category disease or not?

摘要

Background: Significant capillary proliferation is common in post-streptococcal acuteglomerulonephritis (PSAGN) after streptococci and is a prognostic disease. Focalsegmental glomerulosclerosis is a disease characterized by segmental sclerosis although itmay have a poor prognosisCase Presentation: A 50-year-old man with nephrotic syndrome underwent renal biopsy,which showed marked endocapillary proliferation due to enlarged vascular endothelialcells and infiltration of lymphocytes. However, there was no mesangial cell proliferation,mesangial matrix increase, or crescent formation. Electron microscopy showed fusion ofpodocytes without any electron-dense deposits. Immunostaining for CD68 and CD3 waspositive, and the presence of macrophages and T-cells was suggested. Steroid therapy,including pulse therapy, was performed, and then cyclosporine was added to steroidtherapy. Although urinary protein decreased, his renal function did not respond well tosteroid therapy, and the patient initiated dialysis 2 years later.Conclusions: We report this case considering that it was nephritis of unknown originshowing segmental endocapillary proliferation that had not been recognized previously.