Plasma exchange (PEX) is a therapeutic procedure used to treat diseases caused by pathogenic antibodies or immune-complexes through the removal and the replacement of plasma. The frequency of complications and reactions associated with PEX are mild and of limited duration. In systemic autoimmune diseases and in a variety of other conditions, PEX might be use in association with intravenous immunoglobulin (IVIg) or therapeutic monoclonal antibodies for the management of acute or refractory patients to achieve a durable remission.