Management of childhood-onset systemic lupus erythematosus (cSLE) over the last two decades in Spain

Childhood-onset systemic lupus erythematosus (cSLE) is a chronic multisystemic autoimmune disease with a more severe and life-threatening course than SLE in adults. Up to 50-80% of patients have renal or other major-organ involvement, such as in the neurological or nephrological systems, which results in significant morbidity and increased mortality. A search was conducted for lupus-related literature published by Spanish authors in PubMed, Science Direct, MEDES and SciELO databases. The search strategy was based on the keywords "paediatric OR pediatric AND lupus AND Spain" from 2005 to 2024. A manual search was also performed with the above keywords, including "Clinical practice guidelines OR protocols OR recommendations". Significant changes in the management of cSLE have occurred over the last 20 years in Spain. Since there is no consensus among experts at a national scale, the use of off-label drugs and the insufficiency of evidence-based recommendations have become widespread. Antimalarials and glucocorticoids have remained the cornerstone of treatment for several years. However, the side effects of the latter and their association with the accumulation of organ damage have led to the incorporation of immunosuppressants and biologic agents into clinical practice earlier in the course of the disease as glucocorticoids-sparing strategies. Recent advances in cSLE management include the application of the treat-to-target approach and the approval of intravenous belimumab for patients ≥ 5 years, while early results with subcutaneous belimumab in cSLE have as well demonstrated efficacy and safety. Despite the initiation of the Spanish Juvenile Systemic Lupus Erythematosus Registry in 2021, which aims to ascertain the outcomes of cSLE in Spanish patients, a consensus document for the management of these patients in Spain remains elusive. According to Treat to Target TaskGroup, treatment of cSLE should aim to ensure long-term survival and prevent organ damage. The use of glucocorticoid-sparing strategies, including biological agents such as belimumab, represents one of the main current challenges. Indeed, subcutaneous belimumab could be a promising tool in cSLE, as its administration in school-aged patients might reduce school absenteeism and lead to an improvement in the child's overall health.