Updates on Childhood-Onset Systemic Lupus Erythematosus

Systemic Lupus Erythematosus (SLE) is a chronic, autoimmune, and multisystem disease. Childhood-onset SLE (cSLE) contributes up to 20% of all cases of SLE and refers to patients who develop the disease before their 18th anniversary. Impressive discoveries in all aspects of the disease emerge every day; one of the most interesting is whether cSLE is a single or a group of diseases, with diverse physiopathologic processes but sharing a rough phenotype. Patients with early onset disease (<5 years), with associated infections and severe disease manifestations, should urge the possibility of monogenic SLE, which represents a small proportion of all cSLE cases, but often with a more complicated clinical course. Despite its being considered a rare disease, the clinical outcomes could be devastating. Patients with cSLE had higher disease activity indexes than adults. Although the survival has improved, it also implies that patients remain a longer period under the effects of the disease. Enormous advances in the understanding of the physiopathological processes are helping to better diagnose children with lupus; still, we are distant to have a perfectly fitted therapy for all our patients. The outstanding efforts of clinicians and researchers to find new therapeutic strategies are encouraging. In this chapter, you will find a concise description of the novel advancements concerning the disease pathogenesis, classification, assessment of disease activity, treatment, and outcomes.