JUVENILE DERMATOMYOSITIS. WHAT SHOULD AN ADULT RHEUMATOLOGIST REMEMBER?
Summary. Reasoning. Juvenile dermatomyositis (JDM) is a severe rare progressive systemic disease of autoimmune genesis with a predominant lesion of the striated muscles, skin and blood vessels of the microvasculature, which develops in children under 16 years of age, but, despite the severity, mortality from JDM is significantly lower than in adult patients with dermatomyositis/polymyositis, disability of pediatric patients is also twice less common. JDM has certain immunological and clinical differences in the development and course of dermatomyositis in children compared with adults. Methods. On the basis of the literature data, the article presents modern concepts of etiology and pathogenesis, describes the diagnostic criteria for JDM; the comparison of clinical and laboratory manifestations and the frequency of their detection in pediatric and adult patients was carried out, the main differences in differential diagnosis were emphasized. The unified therapeutic strategies are presented according to the recommendations of the international rheumatologic associations. Conclusions. Given current therapeutic options, JDM patients will account for an increasing proportion of patients with inflammatory myositis. This justifies the need for further research on the management of patients with JDM to improve the level of medical care and the long-term consequences of diseases.
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