Proliferative Glomerulonephritis With Fibrils, Monoclonal κ Light Chain, and C3 Deposits
| Autor: | Thomas D. Barbour, Piers Blombery, Guy Touchard, Christophe Sirac, Lucy C. Fox, Moira Finlay, Adam G. Steinberg, Ahida Batrouney, Sébastien Bender, Surender Juneja, Frank Bridoux |
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| Rok vydání: | 2021 |
| Předmět: |
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 |
| Zdroj: | American Journal of Kidney Diseases. 78:459-463 |
| ISSN: | 0272-6386 |
| DOI: | 10.1053/j.ajkd.2021.01.014 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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