Clinical and Epidemiological Characteristics of Scleroderma in Children of the Samara Region: a Single-Stage Retrospective Study

Background . Juvenile scleroderma is an autoimmune disease of connective tissue in children of different ages. Currently, there is no structured information about the course of this disease in children of The Samara Region, which makes it difficult to regularly monitor and timely correct the treatment of the disease. The aim of the study is to create a regional registry, as well as to conduct a clinical and epidemiological analysis of scleroderma in children in the Samara region for 2018–2023. Methods. A single-stage retrospective study was conducted in the period from 2018 to 2023, which included 75 patients treated at the V.P. Polyakov Samara Regional Clinical Cardiology Dispensary, in the Department of Pediatric Cardiac Surgery and Cardiorheumatology with a diagnosis of scleroderma. Results. There has been a 1.3-fold increase in the prevalence of juvenile scleroderma among children (0–17 years old) over the period 2018–2023. In 13% of cases, patients were misdiagnosed, which is probably due to the diversity and non-specificity of the clinical picture of juvenile scleroderma. According to the results of the study, a long diagnostic search can be noted — it took more than two years for 17.3% to make a correct diagnosis, which is most likely due to the lack of an interdisciplinary approach to the management of juvenile scleroderma, as well as the late treatment of parents by a doctor. The complicated course of systemic scleroderma occurred in 1.3% of cases and was characterized by vascular disorders such as nail osteolysis of the distal phalanges, which was associated with an erroneous diagnosis at the outpatient stage and a delayed referral to a rheumatologist. Conclusion. The conducted research allows us to conclude that it is advisable to create a local registry of children with scleroderma, the analysis of which will help pediatricians and rheumatologists of both outpatient and inpatient levels to respond in time to existing deviations in the diagnosis and therapy of scleroderma and optimize approaches to the management of patients with this profile. The limitation of the presented study is the small number of children (n = 75). It can also be assumed that the study did not include all children in the region suffering from scleroderma, given the lack of a registry, which makes it much more difficult to assess the course of the disease.