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Proliferative Glomerulonephritis With Fibrils, Monoclonal κ Light Chain, and C3 Deposits
- Source :
- American Journal of Kidney Diseases. 78:459-463
- Publication Year :
- 2021
- Publisher :
- Elsevier BV, 2021.
-
Abstract
- There is increasing recognition of monoclonal gammopathy as a cause of proliferative glomerulonephritis (GN), including cases in which glomerular deposition of monoclonal immunoglobulin is demonstrated. Recently, proliferative GN with monoclonal immunoglobulin deposits (PGNMID) has incorporated a light chain variant of the disease (termed PGNMID-LC). Intriguingly, glomerular co-deposition of C3 is found in addition to monotypic light chain, implying complement activation via the alternative pathway (AP). We present a unique case of proliferative GN in a 42-year-old man who presented with nephrotic syndrome and was found to have κ light chain multiple myeloma. Immune staining of the glomerulus was positive only for κ light chain and C3, with the striking appearance of nonamyloid fibrils on electron microscopy. Following clonally targeted therapy for myeloma, the renal clinical abnormalities resolved completely. We present detailed molecular studies for light chain and complement and consider local mechanisms whereby monoclonal κ light chain fibrils may have triggered AP activation within the glomerulus.
- Subjects :
- business.industry
030232 urology & nephrology
Glomerulonephritis
Immunoglobulin light chain
Fibril
medicine.disease
Molecular biology
Complement system
03 medical and health sciences
0302 clinical medicine
Nephrology
Monoclonal
Alternative complement pathway
Medicine
030212 general & internal medicine
business
Nephrotic syndrome
Multiple myeloma
Subjects
Details
- ISSN :
- 02726386
- Volume :
- 78
- Database :
- OpenAIRE
- Journal :
- American Journal of Kidney Diseases
- Accession number :
- edsair.doi...........4b7a7dae675f2aea38648b585465309c