Prevalence and burden of pediatric-onset systemic lupus erythematosus.
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease with a highly variable clinical course. Pediatric-onset SLE (pSLE) represents 10-20% of all SLE cases, and is associated with higher disease severity, including more-rapid damage accrual, than adult-onset SLE. As in adults, pSLE disease expression varies according to ethnicity, with a milder disease course in white patients. The majority of pSLE patients will have developed damage within 5-10 years of disease onset, most frequently involving the musculoskeletal, ocular, renal and neuropsychiatric systems. Owing to improvements in disease management and recognition over the past 20-30 years, patients now live longer, but as a result have increased disease damage. Premature atherosclerosis and osteoporosis have become increasingly prevalent morbidities in pSLE patients. Early atherosclerosis leads to a considerable rise in cardiovascular and cerebrovascular events, and failure to develop adequate peak bone mass during adolescence-a crucial period of bone accrual-is likely to lead to early osteoporosis and fractures. Patients with pSLE have an incurable, potentially devastating disease that occurs during a vulnerable period of psychosocial development, leading to specific and unique psychosocial stressors. Additional large, long-term follow-up studies in pSLE are needed to better understand the disease prognosis and to facilitate development of tailored treatments.
Kamphuis
Sylvia
S
Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
Silverman
Earl D
ED
D017668:Age of Onset; D050197:Atherosclerosis / Q000453:epidemiology; D002648:Child; D015897:Comorbidity; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000453:epidemiology* / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D010024:Osteoporosis / Q000453:epidemiology; D015995:Prevalence; D011379:Prognosis; D011788:Quality of Life; D012737:Sex Factors; D015996:Survival Rate