Abstract: TH-PO645
A Case Report of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin G Kappa Deposits in the Setting of Propionibacterium granulosum Infection
Session Information
- Fellows/Residents Case Reports: Genetic Diseases, Pregnancy, Monoclonal Gammopathy
November 02, 2017 | Location: Hall H, Morial Convention Center
Abstract Time: 10:00 AM - 10:00 AM
Category: Nephrology Education
- 1302 Fellows and Residents Case Reports
Authors
- Syeda, Sara, Brown University , Providence, Rhode Island, United States
- Sharif, Sairah, Brown University , Providence, Rhode Island, United States
Background
Proliferative glomerulonephritis with monoclonal immunoglobulin deposition disease (PGNMID) is a rare disease that is caused by monoclonal IgG deposition. On light and electron microscopy it mimics immune complex glomerulonephritis (GN). Here we report a patient with Propionibacterium granulosum (P. granulosum) joint infection who developed acute nephrotic-nephritic syndrome, without detectable paraprotein, eventually found to have PGNMID.
Methods
An 80-year-old Hispanic male with history of insulin dependent diabetes who was being treated with penicillin for left hip P. granulosum prosthetic infection, presented with sudden onset anasarca and serum creatinine (SCr) of 2.8mg/dL (baseline SCr was 0.7mg/dL). Workup showed nephrotic syndrome with microscopic hematuria. Over the span of next two weeks he developed anuric failure requiring dialysis. He underwent a renal biopsy which showed PGNMID on a background of diabetic nephropathy. Immunofluorescence showed 3+ granular staining for IgG, C3 and kappa light chain. Electron microscopy showed diffuse sub-epithelial “humps” and diffuse podocyte foot process effacement. Few mesangial deposits were present, but no sub-endothelial deposits seen. Serology was non-revealing, hepatitis and HIV screens were negative, complement levels (C3, C4) were normal and no paraprotein could be detected in serum or urine. He was started on steroids and given rituximab therapy (4 doses) but he remains dialysis dependent to date.
Conclusion
We report a case of PGNMID in a patient with P. granulosum infection. PGNMID typically presents with nephrotic range proteinuria, hematuria, and renal failure. Approximately one fifth patients progress to end stage renal disease. The pathogenesis is elusive, but one hypothesis is that there is hyper secretion of IgG by plasma cells or a clonal B cell population leading to its glomerular deposition. Only about 30% cases have a monoclonal spike. We speculate that an immune reaction against bacterial antigen may have triggered the process in our case. This may explain widespread sub-epithelial deposits seen on his biopsy, contrary to the literature reports of the deposits being primarily sub-endothelial and mesangial. Overall, PGNMID remains a poorly understood disease and there is no validated optimum therapy for it.
Funding
- Clinical Revenue Support