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

Autor: Isiktas Sayilar E; Department of Nephrology, Ufuk University School of Medicine, Dr. Rıdvan Ege Hospital, Ankara, Turkey., Kiremitci S; Department of Pathology, Ankara University School of Medicine, Ankara, Turkey., Ergun I; Department of Nephrology, Ufuk University School of Medicine, Dr. Rıdvan Ege Hospital, Ankara, Turkey., Ensari A; Department of Pathology, Ankara University School of Medicine, Ankara, Turkey.
Jazyk: angličtina
Zdroj: Nephron [Nephron] 2022; Vol. 146 (4), pp. 377-380. Date of Electronic Publication: 2021 Dec 22.
DOI: 10.1159/000520943
Abstrakt: 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.
(© 2021 S. Karger AG, Basel.)
Databáze: MEDLINE