ABSTRACT

This chapter reviews the mechanisms postulated to account for the benefits of intravenous immunoglobulin (IVIG) infusions in treating autoimmune diseases and as infection prophylaxis in cases of acquired immune deficiencies. There are a limited number of FDA-labeled clinical indications for the use of IVIG in the U.S. : as replacement therapy in primary immunodeficiency diseases; as treatment of idiopathic thrombocytopenia purpura (ITP); and as prophylaxis for bacterial infections in 8-cell chronic lymphocytic leukemia (CLL), for pediatric HIV infection, and for local and systemic infections, interstitial pneumonia, and acute graft vs. host disease (GvHD) after bone marrow transplantation. However, based on the positive clinical experience in the treatment of ITP and Kawasaki disease (KD), IVIG administration has been investigated in a variety of autoimmune diseases and acquired immune deficiencies. (Table 1). Prospective randomized controlled trials are lacking in most of these clinical indications, and the underlying pathophysiological disease processes are not well understood. The mechanisms of action hypothesized to account for the benefits of IVIG administration are largely derived from in vitro data and published reports of small uncontrolled clinical trials and thus may not be applicable to all clinical situations. Nonetheless, our understanding has advanced considerably in the past several years.