Objectives : Intravenous immunoglobulin (IVIG) is applied in the treatment of primary immunodeficiency diseases, autoimmune disorders and inflammatory disorders. The mechanism of anti-inflammatory action of high-dose IVIG is diverse and dependent on the disease entity. The aim of this paper was to define the efficacy and safety of IVIG treatment of rheumatic diseases in children. Material and methods : We performed a retrospective examination of the efficacy of IVIG therapy in pediatric rheumatology. All children were treated in the period 1.01.2009 to 31.12.2013 in the Pediatric Rheumatology Department and Pediatric Department in St. Louis Hospital in Kraków and in the Pediatric Rheumatology Department, Eleonory Reicher Institute of Rheumatology in Warsaw. During 5 years, 70 patients (27 male – 38.57%) received IVIG preparations, 16 of them in concordance with registration recommendations, the others on the basis of reliable published clinical reports. The mean age of children was 6 years, the mean weight was 23.5 kg, and the mean height was 106 cm. Indications for IVIG therapy were: juvenile idiopathic arthritis (JIA), Kawasaki disease (KD), idiopathic thrombocytopenic purpura (ITP), juvenile dermatomyositis (JDM), systemic vasculitis (SV), juvenile lupus erythematosus diseminatus (JLED), scleroderma (SCLE). Statistical analysis was performed using Statistica software version 2.0. Results: All patients experienced a beneficial effect of IVIG adjuvant therapy – clinical improvement and normalization of laboratory tests. There were no adverse effects of this therapy. Conclusions : Autoimmune diseases in children are rare, characterized by diverse clinical course and still unclear pathogenesis. IVIG therapy can be used in pediatric rheumatology as an efficient medication of basic supplementary treatment. Drawing up uniform standards seems to be crucial for IVIG transfusions in pediatric rheumatology, when the results of primary treatment prove ineffective.