Efficacy and Safety of Regular Intravenous Immunoglobulin Replacement Therapy in Primary Immunodeficiency Diseases

Abstract

rimary Immunodeficiency Diseases (PIDs), are inherited disorders that affect the immune system and lead to increased susceptibility to infections, autoimmunity, lymphoproliferation disorders, atopy and malignancy. Intravenous immunoglobulin (IVIG) is being used as antibody replacement therapy in many PIDs including X-linked Agammaglobulinaemia, common variable immunodeficiency, and severe combined immunodeficiency. The optimal dose of IVIG which is required to guard against infection, as well as, the adverse effects that may be encountered in patients with primary immunodeficiency are still under continuous studies. We performed a longitudinal study to evaluate the efficacy and side effects of regular IVIG among patients with primary immunodeficiency diseases. Pediatric patients with primary immunodeficiency diseases including severe combined immunodeficiency (SCID) and predominantly antibody deficiency who require regular IVIG therapy were included in the study. Patients with secondary IgG deficiency and those with hypercoagulable state were excluded from the study Enrolled patients were evaluated initially and periodically for a total duration of one year. IVIG was administered at the standard dose (0.5gm/kg every 4 weeks). Detailed clinical history and clinical examination was performed to determine the demographic data (age, age at onset