Abstract: SA-PO202
Proliferative Glomerulonephritis with Monoclonal IgG Deposits: A Mysterious Renal Disorder
Session Information
- Onconephrology: Immunological Cross-Talk
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Onconephrology
- 1700 Onconephrology
Authors
- Khan, Umair, University of Massachusetts Chan Medical School - Baystate Regional Campus, Springfield, Massachusetts, United States
- Kocas, Zehra Nur, University of Massachusetts Chan Medical School - Baystate Regional Campus, Springfield, Massachusetts, United States
- Papamarkakis, Kostas, University of Massachusetts Chan Medical School - Baystate Regional Campus, Springfield, Massachusetts, United States
Introduction
Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a rare form of glomerulonephritis characterized by glomerular monoclonal deposits. It predominantly presents with nephrotic range proteinuria and renal dysfunction. A third of patients with PGMID progress to ESRD. It is a renal-limited disorder, however, rare cases of PGNMID in association with CLL have been reported. Herein, we present a case of severe renal failure due to PGNMID in the setting of underlying CLL.
Case Description
An 88-year-old female with CLL presented with oliguric renal failure. Labs revealed an elevated serum creatinine level of 7.9 mg/dL (baseline 1.1 mg/dL), and nephrotic range proteinuria. Renal biopsy showed kappa-restricted immune complex-mediated proliferative and crescentic glomerulonephritis (Fig 1) with linear staining of glomerular capillary loops for IgG (3+), and kappa (3+) suggestive of PGNMID.
It was presumed that her PGNMID was due to CLL and hence decision was made to continue with ibrutinib and assess hematological response.
Discussion
PGNMID, a rare form of GN, appears as one of the most mysterious renal disorders associated with monoclonal gammopathy. When present, it should raise the possibility of an underlying secondary etiology as hematological malignancies, especially CLL, have been recognized as a rare cause of PGNMID. Early recognition of PGNMID is a key factor for long term renal survival. Treatment therapy is aimed at monoclonal proliferation and hematological response. Identification of pathogenic clone is vital to guide treatment.
The usual absence of any detectable clonal proliferation makes its management challenging. Further studies in understanding the mechanisms of monoclonal deposition would help refine treatment strategies. This case highlights the importance of close monitoring and individualized treatment plans in the management of PGNMID.