Intravenous immunoglobulins (IVIg) have been widely used in clinical practice for more than 35 years. Their efficacy has been established in many clinical trials for the treatment of autoimmune rheumatic diseases including systemic lupus erythematosus, ANCA positive vasculitis and dermatomyositis, but these indications are classified as the "off label" treatment. For the diseases mentioned above there are no generally accepted therapeutic guidelines. The case reports (one patient with lupus erythematosus chorea, two patients with dermatomyositis and one with the Wegener's granulomatosis) present a treatment of systemic connective tissue diseases with IVIg following the failure of standard therapeutic regimens. A successful therapy has been realized using different doses of IVIg, which raises a question on an appropriate dose. Based on our experience, we conclude that intravenous immunoglobulins are effective in the treatment of many "off label" indications in rheumatology, particularly in cases when standard immunosuppressive therapy could be harmful. Despite the evidence of efficacy, the dosage and timing of IVIg therapy, and questions of costs/benefits ratio still remain insufficiently documented and multicentric controlled clinical trials with consecutive development of guidelines are necessary (Ref. 27).