Prevalence and burden of pediatric-onset systemic lupus erythematosus

Nat Rev Rheumatol. 2010 Sep;6(9):538-46. doi: 10.1038/nrrheum.2010.121. Epub 2010 Aug 3.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Age of Onset
  • Atherosclerosis / epidemiology
  • Child
  • Comorbidity
  • Female
  • Humans
  • Lupus Erythematosus, Systemic / epidemiology*
  • Lupus Erythematosus, Systemic / pathology*
  • Lupus Erythematosus, Systemic / physiopathology
  • Male
  • Osteoporosis / epidemiology
  • Prevalence
  • Prognosis
  • Quality of Life*
  • Sex Factors
  • Survival Rate