Antinephrin-Associated Primary Focal Segmental Glomerulosclerosis Successfully Treated With Plasmapheresis

Minimal change disease and primary focal segmental glomerulosclerosis (FSGS) have similar clinical manifestations, both presenting with rapid-onset nephrotic syndrome and often amendable to treatment with high-dose glucocorticoids. Recently, antinephrin antibodies (Ab) have been reported to be present in a subset of patients with minimal change disease as well as in patients with primary FSGS with recurrent massive proteinuria after kidney transplantation.1-3 Infusion of antinephrin Ab in mice results in rapid-onset proteinuria and subsequent FSGS lesions.