Childhood-onset systemic lupus erythematosus

Childhood-onset systemic lupus erythematosus, abbreviated cSLE and also called juvenile-onset, juvenile or pediatric systemic lupus erythematosus, is a form of the chronic inflammatory autoimmune disease systemic lupus erythematosus that develops in individuals up to 18 years old. Early-onset disease is the term often used for the subset who are up to 5 years old, and these children tend to have a more severe course than those who develop it after 5. It does not include neonatal lupus erythematosus, a lupus-like disease present at birth that is caused by maternal antibodies such as anti-Ro/SS-A and anti-La/SS-B crossing the placenta. Like the adult form, cSLE arises from antibodies that bind antigens in the nuclei and cytoplasm of the person's own cells, and the resulting antibody-antigen complexes trigger uncontrolled inflammation and injury in many tissues and organs. Worldwide prevalence is given as 1.9 to 25.7 per 100,000 children and incidence as 0.3 to 0.9 per 100,000 per year. Although it resembles adult-onset disease, cSLE has a more aggressive onset and course, more frequent exacerbations, more severe organ damage and a higher mortality rate, which makes it a clinical entity distinct from the adult form. It occurs more often in Black, Asian, Hispanic and Native American than in Caucasian populations, and the median age at onset was 12.6 years in a United Kingdom review of 196 cases. Kidney involvement such as lupus nephritis is among the common presentations. This entry records what is known and offers no medical advice.