Membranous Nephropathy with Lambda Light Chain Restriction: A Rare Form with Serum Negative and Tissue Positive PLA2R Ab

Nephron ◽  
2021 ◽  
pp. 1-4
Author(s):  
Emel Isiktas Sayilar ◽  
Saba Kiremitci ◽  
Ihsan Ergun ◽  
Arzu Ensari
Keyword(s):  
Nephrotic Syndrome ◽  
Light Chain ◽  
Membranous Nephropathy ◽  
Capillary Wall ◽  
Glomerular Capillary Wall ◽  
Glomerular Capillary ◽  
Phospholipase A2 Receptor ◽  
Antibody Positivity ◽  
Immunoglobulin Deposits ◽  
Light Chain Deposition

Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in adults. Subepithelial polyclonal immunoglobulin deposits and >70% M-type phospholipase A2 receptor antibody positivity are typical findings in idiopathic MN. A 58-year-old female patient was admitted with clinical presentation of nephrotic syndrome. Autoimmune diseases, infections, and malignancies were ruled out after clinical and laboratory evaluations. Diagnostic work-up revealed serum PLA2R antibody negativity and diffuse thickening of glomerular capillary wall on biopsy, while glomerular capillary wall IgG, C3, and Lambda monotypic light chain deposition and PLA2R1 positivity were detected by immunofluorescence and immunohistochemical examination, respectively. Following prednisolone treatment, creatinine and proteinuria were markedly regressed. The MN cases with a light chain deposits are rare and experience regarding their treatment are insufficient.

scholarly journals Membranous Nephropathy in the Dog

1986 ◽  
Vol 23 (6) ◽  
pp. 718-733 ◽  
Author(s):  
R. S. Jaenke ◽  
T. A. Allen
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Basement Membranes ◽  
Capillary Wall ◽  
Glomerular Capillary Wall ◽  
Renal Lesions ◽  
Pathologic Findings ◽  
Advanced Stages ◽  
Recovery Characteristic ◽  
Immunoglobulin Deposits

Membranous nephropathy, a disease syndrome characterized by severe proteinuria and often accompanied by the nephrotic syndrome, was identified in 29% of a population of 46 proteinuric dogs. Renal lesions were characterized by the presence of subepithelial immunoglobulin deposits distributed diffusely along the glomerular capillary wall. Advanced stages were associated with progressive thickening of capillary basement membranes and incorporation of the immune deposits. These changes were followed by either glomerulosclerosis or recovery. Characteristic morphologic stages were correlated with clinical pathologic findings which showed that the level of proteinuria, hypoalbuminemia, and consequent nephrotic syndrome was most severe in the initial stages of membranous nephropathy while the level of azotemia increased in the more advanced stages of the syndrome.

Very Late Activation-3 Integrin Is the Dominant β1-Integrin on the Glomerular Capillary Wall: An Immunofluorescence Study in Nephrotic Syndrome

Nephron ◽  
1992 ◽  
Vol 62 (4) ◽  
pp. 382-388 ◽  
Author(s):  
Alberto Baraldi ◽  
Luciana Furci ◽  
Giovanna Zambruno ◽  
Elisabetta Rubbiani ◽  
Giorgio Annessi ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Capillary Wall ◽  
Immunofluorescence Study ◽  
Glomerular Capillary Wall ◽  
Glomerular Capillary

scholarly journals Treatment-resistant nephrotic syndrome in dense deposit disease: complement-mediated glomerular capillary wall injury?

2020 ◽  
Vol 35 (9) ◽  
pp. 1791-1795
Author(s):  
Caroline Duineveld ◽  
Eric J. Steenbergen ◽  
Andrew S. Bomback ◽  
Nicole C. A. J. van de Kar ◽  
Jack F. M. Wetzels
Keyword(s):  
Nephrotic Syndrome ◽  
Capillary Wall ◽  
Dense Deposit Disease ◽  
Treatment Resistant ◽  
Glomerular Capillary Wall ◽  
Glomerular Capillary ◽  
Dense Deposit ◽  
Wall Injury

Sialoglycoproteins and filtration barriers in the glomerular capillary wall

1975 ◽  
Vol 51 (3) ◽  
pp. 354-376 ◽  
Author(s):  
Harrison Latta ◽  
William H. Johnston ◽  
Thomas M. Stanley
Keyword(s):  
Capillary Wall ◽  
Glomerular Capillary Wall ◽  
Glomerular Capillary
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