Many primary renal diseases are associated with either antibody deposition within the glomerulus or an antibody associated autoimmunity, as may be seen with certain vasculitidies. Examples of these diseases include Goodpasture's syndrome, cryoglobulinemia, antineutrophil cytoplasmic antibody positive syndromes, and other forms of rapidly progressive glomerulonephritis. Immunoglobulins also may be nephrotoxic to the tubules such as is the case with myeloma related light chains. Given the rapid removal of immunoglobulins by therapeutic plasma exchange, this modality has been considered an appealing management option in the treatment of these renal diseases. Although not classically considered as autoimmune diseases, thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are related syndromes which often involve the kidneys. Although previously unexplained, it has been long appreciated that therapeutic plasma exchange (PE) can be a useful treatment for these microangiopathic hemolytic anemias, but the most recent insights into their pathogenesis suggest that PE may be beneficial by replacing a missing enzyme or removing pathogenic autoantibodies.