Autoimmune mechanisms are involved in the pathogenesis of broad spectrum of renal diseases. Kidney involvement is due to both primary and secondary glomerular disease in native kidneys. Non-immunological causes can inflict serious damage to the kidneys too (e.g., multiple myeloma). In these cases, the efficacy of PEX and other types of apheresis (e.g., immunoadsorption) have been evaluated, in combination with immunosuppressive therapy. Additionally, PEX has been used in kidney transplantation for the management of antibody-mediated rejection and in desensitization protocols. This chapter will present current body of evidence for the effectiveness of PEX in nephrology.