Posttransplant nephrotic syndrome resulting from NELL1-positive membranous nephropathy
Muench J, Krueger BM, Weimann A, Wiech T, Reinhard L, Hoxha E, Pfister F, Halbritter J (2021)
Publication Type: Journal article
Publication year: 2021
Journal
DOI: 10.1111/ajt.16610
Abstract
Membranous nephropathy (MN) constitutes a major cause of nephrotic syndrome (NS) in adults. After kidney transplantation (KTx), both recurrent and de novo MN has been reported. In addition to PLA2R and THSD7A, recent identification of neural EGFL-like-1 protein, NELL1, as a potential disease antigen has enriched our understanding of MN pathogenesis. To date, NELL1-positive MN has only been described in native kidneys, but never been diagnosed in renal allografts. We here report on a 56-year-old male kidney transplant recipient suffering from amyotrophic lateral sclerosis (ALS), who developed NS 25 years after KTx. Allograft biopsy revealed NELL1-positive MN. Using specifically established immunoblotting techniques, we detected new-onset NELL1-IgG1, IgG3, and IgG4 antibodies in the patient ' s serum correlating with the course of proteinuria. While primary renal disease was undetermined, MN recurrence seemed unlikely given the long-time span since KTx. By clinical investigation of de novo etiologies, we did not detect an underlying malignancy. However, previous self-medication with dimercaptopropane sulfonate (DMPS) and alpha lipoic acid (ALA) represented a potential trigger and cessation associated with partial remission of proteinuria. This report illustrates the first case of posttransplant NS due to NELL1-positive MN. Monitoring NELL1 antibodies in the serum promise to be a non-invasive diagnostic tool guiding disease management.
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Germany (DE)How to cite
APA:
Muench, J., Krueger, B.M., Weimann, A., Wiech, T., Reinhard, L., Hoxha, E.,... Halbritter, J. (2021). Posttransplant nephrotic syndrome resulting from NELL1-positive membranous nephropathy. American Journal of Transplantation. https://dx.doi.org/10.1111/ajt.16610
MLA:
Muench, Johannes, et al. "Posttransplant nephrotic syndrome resulting from NELL1-positive membranous nephropathy." American Journal of Transplantation (2021).
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