The autoimmune architecture of childhood idiopathic nephrotic syndrome

Idiopathic nephrotic syndrome (INS), the most common glomerular disorder in children, has long been considered an immune-mediated disease based on the efficacy of glucocorticoids at inducing remission. Nevertheless, the immune processes leading to podocytopathy have largely remained elusive. The success of B cell-depletion with rituximab, descriptions of B cell dysregulation during active disease, and the most recent discovery of autoantibodies targeting the major podocyte antigen Nephrin denote an autoimmune humoral etiology for INS.