Renu Mariam Thomas*
Senior Consultant Histopathologist, VPS Lakeshore, Hospital and Research Centre, Kochi, Kerala, India
Received: 14 November, 2016; Accepted: 08 December, 2016; Published: 09 December, 2016
Dr. Renu Mariam Thomas, Senior Consultant Histopathologist, VPS Lakeshore Hospital and Research Centre, Kochi, India, Tel: 09447196811; E-mail:
Thomas RM (2016) Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposition: Report of Two Cases and Review of Literature. Arch Renal Dis Manag 2(1): 037-039. DOI: 10.17352/2455-5495.000016
© 2016 Thomas RM. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Here we report two cases of proliferative glomerulonephritis with monoclonal IgG deposits, a form of renal involvement by monoclonal gammopathy that mimics immune complex glomerulonephritis. Case 1 presented with incidental proteinuria and a renal biopsy showed mesangioproliferative glomerulonephritis with monoclonal IgG kappa deposits on immunofluorescence examination. He remains stable after one year follow up. Case 2 presented with rapidly progressive renal failure and renal biopsy showed crescentic membranoproliferative glomerulonephritis with monoclonal IgG kappa deposits on immunofluorescence study. He showed no response to aggressive immunosuppressive medication and plasmapheresis and remained anuric. He underwent renal transplantation three months later but disease recurred in the allograft, which was diagnosed on a biopsy one and a half months post-transplant. Extensive work up for underlying paraprotein disease was negative in both patients. Our aim is to expand awareness and highlight the heterogeneity in the clinical presentation, histology and outcome of this rare entity.
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a newly added entity in the spectrum of monoclonal gammopathy associated renal disease. PGNMID resembles immune complex glomerulonephritis (GN) on light (LM) and electron microscopy (EM). It commonly presents with a membranoproliferative pattern and rarely with crescent formation. EM shows granular, non-organized deposits, typically in a sub endothelial and mesangial distribution. The glomerular deposits on immunofluorescence (IF) are monoclonal, staining for a single light-chain isotype and a single heavy-chain subtype, most commonly IgG3. Thirty percent of patients have a detectable circulating monoclonal protein with the same heavy and light chain isotypes as the glomerular deposits; however, the presence of an underlying hematologic malignancy is rare. Patients typically present with nephritic or nephrotic syndrome. The prognosis of this disease is variable. We hereby present two cases of PGNMID with different clinical presentation, pathologic findings and outcome.
42 year old male patient presented with incidentally detected proteinuria. On admission, blood pressure was 140/80 mm Hg. Systemic examination was unremarkable. Laboratory investigations showed hemoglobin 13.6 gm/dl, total leucocyte count 7500/cumm, platelets 2, 84000/cumm, blood urea16mg/dl, serum creatinine 1 mg/dl, serum albumin 3.7gm/dl, sodium/potassium 139/4.6mMol/l. Urine examination showed proteinuria of 1.42 gm/day. Serology for hepatitis B, C and HIV were negative. Serum complement levels were normal. ANA, dsDNA, ANCA, anti GBM antibodies and cryoglobulins were not detected. Ultrasonography showed normal sized isoechoic kidneys. A renal biopsy was done.
Biopsy showed renal cortical tissue with 12 glomeruli, 3 obsolescent (25%). Nonobsolescent glomeruli showed diffuse mild mesangial proliferation (Figure 1) and focal segmental endocapillary proliferation. No double contouring was seen. There were no crescents or tuft necrosis. Interstitium showed focal (1015%) fibrosis and tubular atrophy. Blood vessels were unremarkable.
Immunofluorescence microscopy showed intense (3+) glomerular mesangial granular staining for IgG,C3and less intense (2+) staining for C1q. Kappa light chain was strongly positive in a similar distribution and lambda light chain was negative (Figure 1). IgA and IgM were also negative. These findings led to a diagnosis of PGNMID, IgG kappa. Electron microscopic studies were not done.
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