The purpose of this study is to evaluate the effectiveness of the treatment of juvenile idiopathic arthritis with systemic manifestations with interleukin-1 (IL-1) inhibitors. Material and methods. A prospective analysis of the course of systemic juvenile idiopathic arthritis was performed in a 15-year-old patient; she was examined using laboratory and instrumental methods before and after therapy with the interleukin-1 (IL-1) inhibitor canakinumab. Laboratory examination included general and biochemical blood tests, determination of rheumatoid factor, C-reactive protein, lactate dehydrogenase, procalcitonin, antinuclear antibodies, anti-citrullinated peptide (ACCP) antibody titer, antistreptolysin-O titer. The patient underwent ECG, echocardiography (EchoCG), ultrasound examination of the pleural cavities, abdominal organs, kidneys, knee joints, radiography of the chest cavity organs and radiography of the hand, computed tomography of the chest and abdominal cavities. Results. Examination of the patient revealed: synovitis of the knee joints, bilateral pleurisy and pericarditis, positive markers of the inflammatory process, characteristic of juvenile idiopathic arthritis with systemic manifestations. The patient was diagnosed with leukocytosis up to 20.3×109/l with a stab shift of leukocytes up to 18%, an increase in the erythrocyte sedimentation rate of 55 mm/h, an increase in C-reactive protein up to 288 mg/l, lactate dehydrogenase up to 500 units/l, in the analyzes urine revealed moderate hematuria and proteinuria. X-ray examination revealed structural changes in the knee and interphalangeal joints. We observed the effectiveness of canakinumab therapy. During therapy with canakinumab, after the second injection, positive dynamics was noted: leukocytes 5.1 × 109/l, ESR 6 mm/h, CRP 12 mg/l, relief of symptoms of the disease. Conclusions. During therapy with canakinumab, the patient showed positive clinical and laboratory dynamics of the disease. The study demonstrated the effectiveness of interleukin-1 (IL-1) inhibitors, canakinumab, in achieving remission of the disease.

Rheumatic heart disease (RHD) is a preventable heart disease that remains endemic in developing countries. More than 30 million people in the world suffer from RHD, of which approximately 300,000 die every year, despite the fact that this disease is preventable and treatable. After a period of relative neglect of rheumatic heart diseases due to a decrease in the incidence in developed countries, interest in this problem has increased again over the past decade, due, apparent­ly, to an underestimation of its true prevalence due to the subclinical course of carditis. Research over the past two decades has demonstrated the advantage of diagnosing RHD with echocardiographic screening based on World Heart Federation echocardiographic criteria, which is 10 times greater than the clinical auscultatory picture only and it allowsearly detection of it in patients, while prevention is to be more likely to be effective. Although understanding of the pathogenesis of the disease has advanced in recent years, key issues remain unresolved. Preventing or providing early treatment for streptococcal infections is the most important step in reducing the burden of this disease. The manage­ment of women with rheumatic heart disease before, during and after pregnancy remains a serious task requiring the efforts of a multidisciplinary team. In 2015, a civil society movement was launched aimed at raising awareness and supporting countries seeking to solve the RHD problem. In May 2018, the World Health Organization adopted a resolu­tion aimed at intensifying global and national efforts to prevent and combat acute rheumatic fever/RHD. Ultimately, a combination of treatment options, research and advocacy based on existing knowledge and science provides the best opportunity to cope with the burden of rheumatic heart disease. The article summarizes the latest achievements in the science of RHD and presents priorities for current actions and future research.