Abstract: SA-PO229
Proliferative Glomerulonephritis with Monoclonal IgG Deposits
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 1203 Glomerular Diseases: Clinical, Outcomes, and Trials
Authors
- Shankaranarayanan, Divya, Weill Cornell Medicine, New York City, New York, United States
- Hartono, Choli, Weill Cornell Medicine, New York City, New York, United States
- Lee, Jun B., Weill Cornell Medicine, New York City, New York, United States
- Salvatore, Steven, Weill Cornell Medical College, New York, New York, United States
Introduction
Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a form of monoclonal gammopathy of renal significance (MGRS). We present a case of rapidly progressive glomerulonephritis in a patient with smoldering multiple myeloma (SMM) who responded to steroid pulse in addition to bortezomib and cyclophosphamide.
Case Description
This was a 74-year-old female with SMM who presented with serum creatinine of 3.06mg/dl from 0.9mg/dL one week prior, normal C3/C4, negative cryoglobulin, κ/ λ ratio of 82.6/11.3 (7.29), serum albumin of 2.4g/dl, and no monoclonal spike on serum or urine protein electrophoresis. However, free κ light chains were present on urine immunofixation. The urinalysis revealed 5RBC/HPF and 24-hour urine protein was 3.93g. Bone marrow biopsy done at an outside facility showed κ light chain myeloma with 10-15% marrow involvement. Kidney biopsy (Figure 1.) showed a membranoproliferative pattern of glomerulonephritis with monoclonal IgG-kappa deposits by immunofluorescence and there were 8 glomeruli (of 31) with cellular crescents. No other monoclonal paraprotein related diseases were present. Electron microscopy showed occasional electron dense deposits in the subendothelial space and fine focal mesangial electron dense deposits. The patient was treated with methylprednisolone 500mg daily followed by a prednisone taper and bortezomib twice weekly 1.3mg/m2alternating with cyclophosphamide along with dexamethasone. The serum creatinine had peaked at 6.8mg/dl and improved with treatment to 1.8mg/dl by day 25 of initial presentation
Discussion
First described in 2004, PGNMID is a renal-limited glomerular disease, characterized by paucity of circulating paraproteins with presence of monoclonal IgG (usually IgG3-kappa) deposits in the glomeruli. The optimal treatment for this disease is not known. This case is unique due to rapid renal progression and prompt response to treatment. Considering PGNMID as a differential diagnosis of AKI in a patient with SMM and RPGN “picture” is important since early treatment may alter the renal prognosis in such patients.
Figure-1