Juvenile arthritis with systemic onset (syn: systemic juvenile idiopathic arthritis; SJIA) is the most complex in pathogenesis, severe in course and unfavorable in prognosis variant of juvenile arthritis. The course of SJIA is characterized by the development of severe life-threatening complications, which in the future may lead to the progression of functional and multiple organ failure, delayed physical development and disability of the patient, therefore it is very important to diagnose this disease at an early stage, for which children with suspicion of SJIA must be examined in accordance with the Procedure for providing medical care for children in the profile "Rheumatology" in rheumatology departments of multidisciplinary hospitals, where it is possible to perform the entire complex of diagnostic measures, these are indicated in the clinical recommendations, since at the stage of diagnosis it is very important to exclude diseases appearing in the guise of SJIA, including infectious diseases, These are indicated in the clinical recommendations, because at the stage of diagnosis it is very important to exclude diseases appearing under the guise of SJIA, including infectious, oncologic, oncohematologic, lymphoproliferative, inflammatory bowel, autoinflammatory syndromes and other rheumatic diseases. Federal districts (FD) of the Russian Federation (RF) differ significantly in demographic, climatic, natural-geographical, sociocultural parameters, which cannot but determine differences in the processes of providing medical care to children in the profile of rheumatology. The analysis of these processes is a necessary condition for improving the quality of early diagnosis of juvenile arthritis in pediatric patients. Objective. To carry out a comparative assessment of compliance with clinical recommendations of the scope of examination performed in the Russian Federation at the stage of primary diagnosis of juvenile arthritis with systemic onset, according to the Federal Register of the Ministry of Health of the Russian Federation. Patients and Methods. A retrospective study of discharge records of 927 patients diagnosed with SJIA, receiving genetically engineered biological drugs, included in the Federal Register of persons with hemophilia, cystic fibrosis, pituitary nanism, Gaucher disease, malignant neoplasms of lymphoid, hematopoietic and related tissues, multiple sclerosis, hemolytic-uremic syndrome, juvenile arthritis with systemic onset, mucopolysaccharidosis types I, II and VI, persons after organ and/or tissue transplantation (Register). Results. Our study found that there were significant differences between the Russian Federation regions in the volume of investigations performed at the stage of diagnosis of SJIA and their compliance with clinical recommendations. In all regions of the Russian Federation we found a reliable dynamics of improvement in the quality of examination associated with the improvement of the regulatory and legal framework for the organization of medical care for children in the profile of rheumatology. Conclusion. Our study confirms the need for continuous monitoring of the work of pediatric rheumatology service of the FD, which will optimize the algorithms of early diagnosis of SJIA and other rheumatic diseases in children in order to timely start treatment, including on the basis of the introduction and modernization of digital health technologies, in particular, the system of electronic registers. Key words: Federal Register, juvenile arthritis with systemic onset, diagnosis, federal district, clinical recommendations
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