JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS. WHAT SHOULD AN ADULT RHEUMATOLOGIST REMEMBER?
Summary. Background. Juvenile systemic lupus erythematosus (jSLE) is a severe, rare multisystem autoimmune/autoinflammatory disease with symptoms onset before the age of 18, which affects any organ system, leads to organ damage, resulting in disability and /or death. Patients with jSLE present 15–20% among patients with systemic lupus erythematosus (SLE). Methods. The article presents current concept of the etiology and pathogenesis, describes the heterogeneity of clinical manifestations and the disease course in jSLE; highlights the main difficulties of diagnosis, in particular the development of diagnostic criteria, based on the latest literature data. The comparative characteristic of the existing classification criteria in the population of jSLE patients is described. Therapeutic strategies for jSLE are predominantly extrapolated from the adult population of SLE patients. The modern therapeutic strategy for the management of jSLE patients, including the use of biological agents is presented. Consclusions. The multisystem nature of the disease, typical for jSLE, the importance of the patient management with the involvement of a multidisciplinary team, not only a pediatric rheumatologist, requires adherence to the established principles of continuity with the adult rheumatology care during the transition of jSLE patient.
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