Systemic lupus erythematosus in children
An estimated 15%-20% of all systemic lupus erythematosus (SLE) is diagnosed during childhood (childhood-onset systemic lupus erythematosus [cSLE]). This chapter focuses on the unique features of cSLE, including clinical presentation and manifestations, treatment, and outcomes, highlighting differences from adult-onset disease. Perhaps the most striking difference is the more severe phenotype that occurs with earlier age of onset. Monogenic causes of SLE are rare in adult-onset disease but are increasingly recognized as mechanistic drivers of disease in the youngest patients. Treatment considerations unique to pediatric patients include the effects of medicine on growth and development, as well as a more limited evidence base to guide treatment, as clinical trials rarely focus on cSLE patients. Clinicians, patients, and families also face challenges in medical decision-making trying to balance the long-term effects of medication with projected disease damage based on limited data, due to the difficulty of studying long-term outcomes after patients transition to adult care.
