Systemic juvenile idiopathic arthritis in the pediatric practice of Donetsk region

Abstract

Juvenile idiopathic arthritis with systemic onset is a special, rare and the most severe variant of juvenile idiopathic arthritis. The article analyzes the clinical features of the onset and course of juvenile idiopathic arthritis with systemic onset in children living in the ecologically disadvantaged Donetsk region and the efficacy of therapy. Clinical cases are described. According to its clinical manifestations, the systemic variant of juvenile idiopathic arthritis is characterized by the severity of the general inflammatory response, a bright clinical picture, severe damage to internal organs, the development of polyserositis, can lead to the development of life-threatening conditions, such as macrophage activation syndrome, and also leads to the formation of deforming arthritis with early disability of the sick child. The relevance of studying the problem of juvenile idiopathic arthritis with a systemic onset is related to the late diagnosis of the disease, because at the onset of the disease there may be no joint syndrome, and therefore it is impossible to use the criteria of the International League of Rheumatology Associations to verify the diagnosis, which leads to diagnostic errors. Nonsteroidal anti-inflammatory drugs, glucocorticosteroids, and immunosuppressants are used for treatment. The prescription of genetic engineering biological therapy, the choice of the drug, is carried out according to the recommendations of the American College of Rheumatology, depending on the preference for systemic or joint manifestations of the disease. But the question of the optimal approach to regimens of dose reduction, duration of biological therapy, and rules for its withdrawal remains open. Until now, the optimal approach to the treatment of juvenile idiopathic arthritis with a systemic onset is unknown. To date, the issue of treatment in patients with pharmacoresistant variants and persistent course of the disease has not been solved. Therefore, further in-depth study of this problem, optimization of the diagnostic algorithm and an individual approach to therapy are needed.