Characteristics of systemic juvenile idiopathic arthritis in patients receiving biologicals: the results of the analysis of the Federal Russian Register

Background. A register is a critical component of the healthcare system. It consists of the informational part (number of patients, spectrum and severity of pathology, dynamics of each patient's condition, administration of specific drugs, and therapeutic effects) and an analytical part (treatment efficacy, planning individual and regional needs for specific drugs and therapeutic effects, development and evaluation of management protocols, etc.). In Russia, despite the existence of the National Federal Register, the number of parameters collected is limited; the Register lacks information about the disease course, clinical characteristics of patients, outcomes, and treatment costs. Thus, the existing shortcomings of the Federal Registry do not allow us to address the problems related to the management of the healthcare quality for patients with systemic juvenile idiopathic arthritis (sJIA). Objective. To analyze geographical, demographic, and clinical characteristics of sJIA patients receiving biologicals, time to diagnosis, and time to admission to a Federal Center according to the Federal Register of the Russian Federation. Patients and methods. We conducted a retrospective study that included 927 sJIA patients receiving biologicals according to the Federal Register. Results. The majority of patients resided in the Central and Volga Federal Districts (221 (23.8%) and 206 (22.3%), respectively). Half of the patients (n = 472 (50.8%)) had their disease onset before the age of 5 years. The following symptoms were registered at disease onset: fever (n = 908 (98%)), rash (n = 748 (80.7%)), enlarged liver and/or spleen (n = 562 (60.6%)), and serositis (n = 198 (21.4%)). A total of 708 children (76.4%) had active arthritis at the moment of disease onset, whereas 130 patients developed arthritis later. Median time to the development of articular syndrome was 24 months. Median time between the disease onset and the diagnosis was 2.93 months. Median time between the first symptoms and admission to a Federal Center was 6.7 months. Conclusion. Analysis of data from the Federal Register of sJIA patients receiving biologicals demonstrated that sJIA in the Russian population is characterized by an acute and early onset with severe extra-articular manifestations, and articular syndrome. The diagnosis was usually verified and the patients were hospitalized to specialized rheumatology departments of Federal Centers within 5 months. Key words: Federal Register, systemic juvenile idiopathic arthritis, biological, disease course