Related Experiment Videos
[Membranoproliferative glomerulonephritis-like lesion with fibrillary deposition associated with multicentric
H Akimoto1, M Shirai, S Usutani
1Department of Internal Medicine, Teikyo University School of Medicine, Tokyo, Japan.
Abstract:
We report a case of a 65-year-old man presenting with multicentric Castleman's disease (MCD) accompanied by membranoproliferative glomerulonephritis-like lesion with fibrillary deposits. The lesion was characterized by highly organized ultrastructual deposits that were negative for Congo-red stain and for immunoglobulin, light chain and C3. Thus, this renal lesion was considered histologically to be fibrillary glomerulonephritis presenting by light microscopy as mesangiocapillary glomerulonephritis. To our knowledge, among the limited number of cases of renal lesion associated with MCD ever reported, this is the first case of a biopsy-proven fibrillary glomerulonephritis. Serum interleukin 6 (IL-6), known as an indicator of MCD activity and as an autocrine growth factor for mesangial cells, was chronologically measured. Augmentation of urinary IL-6 simultaneously with that of extra renal symptoms of MCD and associated renal disease may indicate an underlying role of this cytokine in the present case. Failure to detect of IL-6 in the glomeruli may support the notion that IL-6 is derived from extrarenal lymphonodi, and not to an in situ product of the glomeruli. However, it may have been related to glomerular injury.
Insights
This study details a rare case of fibrillary glomerulonephritis in a patient with multicentric Castleman's disease (MCD). Elevated Interleukin-6 (IL-6) levels correlated with disease activity, suggesting its role in MCD-associated kidney injury.
Area of Science:
- Nephrology
- Oncology
- Immunology
Background:
- Multicentric Castleman's disease (MCD) is a rare lymphoproliferative disorder.
- Renal involvement in MCD is uncommon, with limited reported cases.
- Fibrillary glomerulonephritis (FGN) is a distinct glomerular disease characterized by fibrillary deposits.
Observation:
- A 65-year-old man with MCD presented with membranoproliferative glomerulonephritis-like renal lesions.
- Biopsy revealed organized ultrastructural deposits, negative for Congo-red, immunoglobulin, light chain, and C3, consistent with FGN.
- This represents the first biopsy-proven case of FGN associated with MCD.
Findings:
- Serum and urinary Interleukin-6 (IL-6) levels were monitored.
- Increased urinary IL-6 correlated with MCD and renal disease exacerbations.
- IL-6 was not detected in glomeruli, suggesting extrarenal origins.
Implications:
- This case highlights a novel association between MCD and FGN.
- IL-6 may play a significant role in the pathogenesis of MCD-associated renal complications.
- Further research is needed to elucidate the precise mechanisms linking IL-6, MCD, and FGN.