TY - CHAP M1 - Book, Section TI - Systemic Lupus Erythematosus, Overlap Connective Tissue Diseases, and Mixed Connective Tissue Disease A1 - Hiraki, Linda Tayeko A1 - Silverman, Earl Dean A2 - Kline, Mark W. Y1 - 2018 N1 - T2 - Rudolph's Pediatrics, 23e AB - Systemic lupus erythematosus (SLE) represents the prototype of autoimmune disease, with the presence of autoantibodies as its hallmark. The incidence of SLE diagnosed in patients younger than age 16 is approximately 0.25 to 11 new cases per 100,000 annually, with an overall prevalence of 1.89 to 63.1 per 100,000 depending on ascertainment and ethnicity of the population. The prevalence and incidence rates are higher in the 12- to 18-year age group. Both the incidence and prevalence rates are 2.4 to 4.9 times higher in African Americans, Native Americans, Asians, Southeast Asians, and Hispanics compared with white children. The female predominance (4–5:1) in pediatric patients is lower than that in adults (9:1), although some but not all studies suggest that the female-to-male ratio may vary in different ethnic populations. The mean age at diagnosis is approximately 12 to 13 years, but presentation of patients as young as age 5 years is routinely reported, and the age of onset varies by ethnicity, with nonwhite populations tending to have onset of disease at younger ages. Presentation prior to age 3 to 5 years is frequently associated with a genetic defect. Despite significant improvements in the outcome of childhood-onset SLE (cSLE), mortality rates remain substantial, with a United Kingdom study reporting standardized mortality ratios (compared to national statistics in the general population) as high as 18.3 for all cSLE and 87 for those with SLE onset at <10 years of age (however, the confidence intervals were wide). SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/16 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1182932412 ER -