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This review summarizes the clinical evidence and practical details for the use of plasmapheresis and other apheresis modalities for each indication in nephrology. Updated information on the molecular biology and immunology of each renal disease is discussed in relation to the rationale for apheresis therapy and its place amid other available treatments. Autoantibody-mediated diseases, such as anti-GBM (anti-glomerular basement membrane) glomerulonephritis (GN), ANCA (antineutrophil cytoplasmic antibody)-related GN and the antibody-mediated type of TTP (thrombotic thrombocytopenic purpura), and alloantibody-mediated diseases such as kidney transplant sensitization and humoral rejection, can be treated by various plasmapheresis methods. These include standard plasmapheresis with a replacement volume, or plasmapheresis with online plasma purification using adsorption columns or secondary filtration. However, it should be noted that the pathogenic molecules implicated in FSGS (focal segmental glomerulosclerosis), myeloma cast nephropathy, and perhaps other diseases are too small to be removed by most online purification methods. A great majority of controlled trials and series on which evidence-based treatment recommendations are made were performed using centrifugal plasmapheresis; it is presumed that membrane-separation plasmapheresis is equally efficacious. For some rarer diseases, such as MPGN (membranoproliferative GN) type 2 with factor H abnormalities or C3Nef (C3 nephritic factor) autoantibodies, there are only a few case reports, but enough scientific understanding to warrant a trial of plasmapheresis in severe cases. Photopheresis, which is effective for cell-mediated rejection in heart and lung transplantation, has not yet found a place in the routine treatment of kidney transplant rejection.