The main views on one of the most complex pathological conditions in pediatric rheumatology, namely systemic arthritis, are presented. The characteristics of the classification positions of this disease are given, the pathogenetic bases, topical at the present time, the main approaches to its therapy are covered. Data from recent studies suggest that systemic arthritis belongs to a separate nosological form in the structure of juvenile idiopathic arthritis, with the development of activation of innate immunity, the presence of genetic determination and the occurrence of a cytokine imbalance with the prevalence of interleukins 1 and 6. Management of systemic arthritis requires aggressive and sufficiently rapid therapy with application of high-dose glucocorticoids and biological therapy. This disease is characterized by the development of a wide range of complications, among which a life-threatening condition — the macrophage activation syndrome deserves special attention. Early effective treatment is highly desirable, since there is a window of opportunity to stop the pathological reaction of the immune system and to prevent the further development of the disease. The overall strategy for the treatment of systemic arthritis is more important than individual therapeutic agents. The structure of systemic arthritis therapy may be wider than it is described in current recommendations and protocols.