Proliferative glomerulonephritis with monoclonal IgG deposits a case report

Proliferative glomerulonephritis with monoclonal immunoglobulin deposit disease (PGMID) is a novel form of glomerular injury due to monoclonal IgG deposition described recently. The disease usually presents as nephritic or nephrotic syndrome with or without renal insufficiency. Histologically, it often presents as membranoproliferative glomerulonephritis (MPGN), followed by endocapillary proliferative glomerulonephritis (GN) and membranous nephropathy(MN). Immune deposits exhibits monoclonal immunoglobulin G(IgG), mostly IgG3 and rarely IgG2. Prognosis of the disease is variable with significant proportion of patient reaching end stage senal disease (ESRD) in few years. Currently, the treatment of PGNMID is empirical and based on individual case reports. We reported a case of PGNMID. The patient was a 27 years old woman presented with nephrotic proteinuria, heamaturia and renal insufficiency. Serological and rheumatological workup were normal. Renal biopsy revealed MPGN pattern of injury with monoclonal IgG immune deposits. Electron microscopy showed granular subendothelial and mesangial deposits. She had no evidence of paraproteinemia in urine and serum and her bone marrow examination was normal. She was treated with predinisolone and rituximab. Despite treatment, she progressed to ESRD and became dialysis dependent. Renal transplantation is the best form of renal replacement therapy (RRT) in ESRD patients though there are few case reports of recurrent PGNMID in the post-transplant patients. Our patient is on live related renal transplant program with her sister being the prospective donor. Feature randomized controlled studies are needed to define the optimal treatment and to assess the long term outcome of this rare entity.