A New Hope for Treating Podocytopathies — Emerging Role of Anti-Nephrin Antibody

The etiology of primary podocytopathies including childhood nephrotic syndrome, minimal change disease, primary focal segmental glomerulosclerosis as well as recurrent focal segmental glomerulosclerosis had long remained elusive. The development of robust anti-nephrin autoantibody detection methods, the identification of these antibodies in idiopathic nephrotic syndrome, and the demonstration of their causal role in podocytopathy have led to a paradigm shift in our understanding of these diseases, offering new insights into pathophysiology and promising improved diagnostic, prognostic, and therapeutic approaches. Prognostication and risk stratification based on anti-nephrin antibody detection as well as personalized therapeutic strategies including therapies targeting antibody-producing B cells or even antigen-specific depletion of autoreactive B cells could markedly improve treatment outcomes and patient quality of life in the future. In this review, we explore the prevalence of anti-nephrin antibodies in various glomerular diseases, current treatment options for primary podocytopathies, and how the emerging evidence of anti-nephrin antibody-mediated podocytopathy could influence future treatment approaches for affected patients.