Abstract: TH-PO0804
Grossly Interesting: An Unusual Presentation of Proliferative Glomerulonephritis with Monoclonal Immunoglobulin Deposits
Session Information
- Glomerular Case Reports: Membranous, PGN, GBM, and More
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Quintero, Sofia, University of Nebraska Medical Center Department of Internal Medicine, Omaha, Nebraska, United States
- McMillan, David A., University of Nebraska Medical Center Department of Internal Medicine, Omaha, Nebraska, United States
- Foster, Kirk W., University of Nebraska Medical Center, Omaha, Nebraska, United States
- Ravipati, Prasanth, University of Nebraska Medical Center Department of Internal Medicine, Omaha, Nebraska, United States
- Mullane, Ryan, University of Nebraska Medical Center Department of Internal Medicine, Omaha, Nebraska, United States
Introduction
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is an uncommon disease with varying clinical presentation but typically presents in older adults with proteinuria and kidney dysfunction. Here, we describe an atypical case wherein a young woman presented with persistent gross hematuria, highlighting the heterogeneity in presentation of PGNMID.
Case Description
A 23-year-old female presented with several months of painless gross hematuria. Urologic evaluation was negative, and she completed antibiotic therapy for presumed urinary tract infection; however, her gross hematuria was persistent despite not actively menstruating, and she later developed facial and lower extremity swelling. Evaluation showed normal kidney function (serum creatinine (sCr) 0.7 mg/dL), nephrotic range proteinuria (13.73 g/24 hours), and low C3. Kidney biopsy demonstrated proliferative glomerulonephritis with immunofluorescence positive for C3 and kappa-restricted IgG. Electron microscopy revealed electron-dense deposits in mesangial and sub-endothelial areas. Thus, IgG subclass staining was pursued, and it showed monotypic IgG3, confirming the diagnosis of PGNMID. Hematologic evaluation failed to detect a clonal population. The patient was initiated on plasma-cell directed therapy with daratumumab, bortezomib, cyclophosphamide, and dexamethasone. Six months post-treatment, she had resolution of gross hematuria and edema, improvement in proteinuria to 2.3g/24 hours, and stable kidney function (sCr 0.8 mg/dL).
Discussion
Gross hematuria is commonly associated with structural or infectious genitourinary disease, but it can be a presenting sign of glomerular disease. Ensuring the resolution of hematuria if attributed to infection or menstruation is key. Here, continued follow-up of persistent hematuria led to evaluation for glomerular disease and ultimately, the correct diagnosis. PGNMID is uncommon in young women, and outcomes are generally poor, with most patients being unable to achieve complete remission. Observational data suggest a clone-directed therapeutic approach may improve outcomes; however, high-quality randomized trials are lacking. In this case, plasma-cell directed therapy led to resolution of symptoms, preservation of kidney function, and substantially improved proteinuria.