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Prevalence and burden of pediatric-onset systemic lupus erythematosus

Nature Reviews Rheumatology volume 6, pages 538–546 (2010)Cite this article

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Abstract

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.

Key Points

  • Pediatric-onset systemic lupus erythematosus (pSLE) follows a more severe clinical course than adult-onset SLE, with higher disease activity and more-rapid damage accrual

  • Despite improved disease management, a considerable proportion of pSLE patients still die before the age of 30 years

  • Premature atherosclerosis and bone-related complications, including osteoporosis and osteonecrosis, have become important morbidities in SLE

  • Optimal control of disease activity is necessary to minimize the risk for damage secondary to disease and therapy-related complications and to decrease mortality

  • Disease expression and outcome differs between white and non-white patients with pSLE, but larger studies are needed to verify the effect of ethnicity on disease course and outcome

  • Transfer of adolescents with pSLE to a dedicated rheumatologist with specific knowledge of the characteristics of pSLE and the unique needs of these patients is essential to achieving good long-term outcomes

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Acknowledgements

S. Kamphuis is supported by the Sophia Children's Hospital Foundation and the Dutch Arthritis Foundation. She is an Assistant Professor in Rheumatology at the Sophia Children's Hospital, Erasmus Medical Center Rotterdam, Netherlands, currently on leave of absence. E. D. Silverman holds the Ho Family Chair in Pediatric Autoimmunity and is supported by grants from the Heart and Stroke Foundation of Ontario, Canada.

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  1. Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, M5G 1X8, ON, Canada

    Sylvia Kamphuis & Earl D. Silverman

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  1. Sylvia Kamphuis
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S. Kamphuis and E. D. Silverman contributed equally to researching data for the article, discussing the content, writing the article, and reviewing and/or editing the manuscript before submission.

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Correspondence to Earl D. Silverman.

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Kamphuis, S., Silverman, E. Prevalence and burden of pediatric-onset systemic lupus erythematosus. Nat Rev Rheumatol 6, 538–546 (2010). https://doi.org/10.1038/nrrheum.2010.121

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