Key aspects for an adequate immunoglobulin therapy of primary immunodeficiencies

Abstract

Over the last two decades we have learned some things about adequate immunoglobulin therapy in immune deficiency. However, there is still a lack of understanding about many of the aspects of this form of treatment as, for example, autoimmunity. There is convincing evidence showing that immunoglobulin therapy in immune deficiency is not a mere passive transfer of antibodies to simply neutralize pathogens. For a basic scientist or an immunologist viewpoint, immunoglobulin is a complex pharmacological component, a therapeutic pool of natural polyspecific, polyreactive antibodies from several thousand healthy donors. This large repertoire of reliable origin may explain the immunomodulatory functions in an autoimmune situation. Autoimmunity is one of the most important immune dysregulations present in primary immune deficiency. The pathogenesis of autoimmunity in immune deficiency is, for the most part, unclear. Treatment with high-dose immune globulin (IVIG) and/or steroids can be helpful. Controlled trials have proved that the administration of high-dose IVIG is adequate to treat patients with persistent primary immune thrombocytopenia (ITP), a common acquired autoimmune disorder characterized by decreased platelet count. Moreover, in the past decades, replacement IVIG therapy has become the standard of care in patients with primary and secondary antibody defects. Attention is turning more progressively to the increasing number of patients on this therapy and diversity of physicians who care for them. In the Symposium organized on the occasion of the XIV Congress of the European Society for Immunodeficiencies (ESID), which took place in Istanbul, Turkey on 13 October 2010, all these particularly relevant aspects of immunoglobulin therapy in the treatment of immune deficiency were focused upon.