Diagnosis and management of juvenile-onset SLE

Systemic lupus erythematous (SLE) is a rare, multi-system, autoimmune disorder. Juvenile-onset SLE (JSLE) differs from the adult form in terms of severity, variation in organ involvement and gender ratio. As well as being a challenging diagnosis to make in the younger age group, the management of JSLE compared to adult-onset SLE requires special consideration towards both the significant long-term consequences of the disease, and its onset during a crucial time in growth and development. The variety of genetic, autoantibody and host immune responses featuring in the disease results in a clinically heterogeneous phenotype. A personalized approach is required to provide optimal care for an individual’s needs and therefore an overview of generic rather than specific management guidelines are presented here.A comprehensive, multi-disciplinary team approach to the management of JSLE is crucial. To date, clinical trials informing interventions in JSLE are very limited. Trials in adult-onset SLE have informed much of the treatment of JSLE. Patient and family involvement in research to improve outcomes is essential in JSLE. New therapies, including biological therapies, are becoming available. New treatment combinations have been used to induce and maintain clinical remission. In order to develop novel therapeutic targets to improve patient outcome, further investigations of the genetic and immune pathways involved in the pathogenesis of JSLE are needed.