Paediatric or juvenile-onset systemic lupus erythematosus (JSLE) is a rare autoimmune condition, differing from the adult form in terms of severity, organ manifestations, and a less striking female predominance. The diagnosis relies on the adult-derived American College of Rheumatology SLE classification criteria. Genetic, autoantibody, and host immune responses, characteristic of this disease, result in a clinically heterogeneous phenotype. A proportion of paediatric SLE patients will have evidence of a genetic deficiency known to be associated with SLE, such as C1q deficiency, and screening for these is required. A challenging diagnosis to make in the younger age group, the management of JSLE compared to adult-onset SLE requires special consideration towards the significant long-term consequences of the disease and treatment toxicity, combined with an onset during a fundamental time with regards to growth and development. A comprehensive, multidisciplinary team approach to the management of JSLE is essential. With a more severe phenotype and limited comorbidities, patients with JSLE represent an invaluable opportunity for investigating the pathogenesis. To date, clinical trials informing interventions in JSLE are very limited and treatment choices rely on the outcome of adult trials. Patient and family involvement in research to improve outcomes and understanding is essential. New treatments, including biological therapies, are becoming available for clinical use and new treatment combinations have been used to induce and maintain clinical remission.
Neutrophil cytosolic factor 2 (
NCF2
) gene polymorphism is associated with juvenile‐onset systemic lupus erythematosus, but probably not with other autoimmune rheumatic diseases in children
