Journal
MEDICINA CLINICA
Volume 157, Issue 3, Pages 121-129Publisher
ELSEVIER ESPANA SLU
DOI: 10.1016/j.medcli.2021.02.010
Keywords
Membranous nephropathy; Nephrotic syndrome; Anti-PLA2R antibodies; Immunosuppressive therapy; Anti-CD20 treatment
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Primary membranous nephropathy is an autoimmune kidney disease and the most common cause of nephrotic syndrome in adults, with the majority of cases caused by antibodies. Recent studies have shown that biological agents targeting autoantibody-producing cells are effective in controlling the disease. This review summarizes key concepts about the pathogenesis, diagnosis, treatment, and monitoring of primary membranous nephropathy.
Primary membranous nephropathy is an autoimmune kidney disease and the most common cause of nephrotic syndrome in adults. About 70%-80% of cases are caused by anti-PLA2R antibodies. Its association with anti-THSD7A antibodies and other autoantibodies has also been described. Recent pilot studies and clinical trials have shown that several biological agents targeting autoantibody-producing cells are effective in controlling the disease with an acceptable safety profile. In this narrative review, we update key concepts about the pathogenesis, autoantibody-based diagnosis, and kidney biopsy findings in primary membranous nephropathy. In addition, we propose a diagnostic and therapeutic algorithm, including guidance on monitoring the response to therapy. We compare the efficacy and safety of currently available treatments, including rituximab and new biological agents, and identify unmet clinical needs. (c) 2021 Elsevier Espana, S.L.U. All rights reserved.
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