«Портрет» пациента с ювенильным артритом: результаты Всероссийского когортного ретроспективного исследования
Juvenile arthritis (JA) is a group of clinically heterogeneous arthritis developing in children under 16 years of age, with a duration of at least 6 weeks, if other joint pathology is excluded. Worldwide, ~2 million children and young people suffer from JA, which allows us to consider it the most common rheumatic disease (RD) in children and choose it as a model for studying the peculiarities of the organisation of medical care for children with RD in order to develop a set of measures to improve its quality and accessibility. The study of the peculiarities of the organisation of medical care for children should include an analysis of clinical manifestations at the onset of the disease, the so-called ‘portrait’ of the patient. The description of the patient's ‘portrait’ for each variant of JA can optimise diagnostic processes, in particular, reduce the patient's ‘pathway’ from disease onset to diagnosis verification and prescription of pathogenetic therapy, which determines the relevance of the present study. Objective. To identify the peculiarities of clinical manifestations (‘portrait’) of juvenile arthritis variants in the Russian Federation. Materials and methods. We conducted a cohort retrospective observational study – analysis of discharge epicrises of 2530 patients with SA living in 8 federal districts, in 84/89 (94%) subjects of the Russian Federation, diagnosed at the age of 0 to 18 years: 1349/2530 (53%) patients were observed in the rheumatology department of the Federal State Institution ‘Children's Health Center’ of the Ministry of Health of Russia, 1181/2530 (47%) – in medical organisations of the subjects of the Russian Federation. Statistical processing of the obtained data was performed using the R-Studio software package version 2022.12.0 (Posit Software, PBC). Results. A total of 702/2530 (28%) and 681/2530 (27%) patients were observed with diagnoses of ‘pauciarticular juvenile arthritis’, ‘juvenile polyarthritis (seronegative)’, respectively. A smaller proportion of the total cohort is represented by patients with JA with systemic onset, juvenile ankylosing spondylitis, juvenile rheumatoid arthritis and JA with psoriasis – 420/2530 (17%), 351/2530 (14%), 200/2530 (8%) and 176/2530 (7%) patients, respectively. Majority of the patients were female – 1585/2530 (63%). 1127/2530 (45%) of the 2530 patients in our sample live in Central Russia – in the Central and Volga Federal Districts – 618/2530 (25%), 509/2530 (20%) patients, respectively. 1403/2530 (55%) patients reside in the Northwestern, Siberian, Ural, Far Eastern, Southern, and North Caucasian federal districts. JA debuted predominantly between 5 and 10 years of age (in 942/2530 (37%) patients), the age of debut of the different variants of JA differing (p = 0.000). The most frequent trigger factor associated with the debut of JA is an infectious disease (p = 0.000). A common symptom for all variants is joint involvement – arthritis at disease debut in the overall cohort was reported in 2349/2530 (92,8%) patients (p = 0.06). Other clinical manifestations in the disease debut are characterised by a different degree of prevalence depending on the variant of JA. ‘Portrait’ of a patient with JA at the onset of the disease, observed in a medical organisation of federal subordination, is characterised by more vivid clinical symptoms, pronounced inflammatory activity. Conclusion. Objective analysis of medical records is a key source of information about the peculiarities of the ‘portrait’ of a patient with JA in the Russian Federation. The identified features of the course of the disease must be taken into account in the development of a set of measures to optimise the processes of medical care for children with JA. The presented data on the ‘portrait’ of the patient should be taken into account in the development of additional education programmes in paediatric rheumatology in order to increase the alertness of – specialists of the first contact, including rheumatologists, with regard to the possible debut of JA in patients with joints, eyes, fever, spotty-papular or linear rash, psoriasis, back pain, which will reduce the time of diagnosis and the start of treatment and, consequently, prevent irreversible damage to organs and systems, and reduce the risk of disability. Key words: juvenile arthritis, juvenile arthritis, “portrait” of a patient, clinical manifestations of juvenile (juvenile) arthritis, medical care for children with rheumatic diseases
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