JUVENILE SCLERODERMIA — PROBLEM ASPECTS
Summary. Introduction. Juvenile scleroderma, with its two varieties, juvenile localized scleroderma and systemic sclerosis, is the third most common rheumatic disease in childhood. In recent years, there has been a qualitative approach to the diagnosis, therapy, and follow-up of children with scleroderma, based on the best available evidence and in accordance with guidelines for evaluation and management of treatment with accrual of improved outcomes. Methods. Scientific data on modern manifestations and changes in views on certain positions of etiology and pathogenesis, diagnostic criteria, clinical manifestations, classification parameters of scleroderma related to pediatric and adult rheumatology are analyzed. Results. The problems of the current state are analyzed, attention is focused not only on the assessment of the skin condition, but also on the early signs of changes in internal organs, the correct treatment of which can significantly affect the long-term result. A pediatric assessment of disease severity using a multidimensional severity scale called «J4S» is presented, which includes parameters of growth, skin, and internal organ damage. A step-by-step approach to treatment with the unification of medical care standards has been defined, which facilitates decision-making in the full practice. Changes in the management of localized scleroderma, where clinical and instrumental assessment systems are more widely used, are distinguished. Consensus confirmation based on long-term studies of the important role of methotrexate in treatment is shown. Conclusion. The implementation of the latest recommendations for the diagnosis and treatment of localized and systemic forms of scleroderma in children is necessary to increase the effectiveness and correct treatment, which can significantly affect the long-term outcome, reducing morbidity and mortality from this disease. Cooperation between adult and pediatric rheumatologists on the basis of evidence-based medicine remains important to store the treatment strategy and follow-up of sick children.
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