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 FSGS as well as recurrent FSGS 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 on the basis of 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.