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The bm12 Inducible Model of Systemic Lupus Erythematosus (SLE) in C57BL/6 Mice
Published on: November 1, 2015
Prevalence and burden of pediatric-onset systemic lupus erythematosus
Sylvia Kamphuis1, Earl D Silverman
1Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, ON M5G 1X8, Canada.
Insights
Pediatric-onset systemic lupus erythematosus (pSLE) causes significant organ damage and unique psychosocial challenges in children. Long-term studies are crucial for understanding pSLE prognosis and developing targeted treatments.
Area of Science:
- Rheumatology
- Pediatric Autoimmunity
- Chronic Disease Management
Background:
- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with variable presentation.
- Pediatric-onset SLE (pSLE) accounts for 10-20% of cases and exhibits greater severity and faster damage accrual than adult-onset SLE.
- Disease expression in pSLE varies by ethnicity, with white patients often experiencing a milder course.
Purpose of the Study:
- To summarize the clinical course, damage patterns, and morbidities associated with pediatric-onset SLE.
- To highlight the long-term consequences and psychosocial impact of pSLE.
- To emphasize the need for further research in pSLE.
Main Methods:
- Review of existing literature on pediatric-onset SLE.
- Analysis of disease progression, damage accrual, and associated morbidities.
- Examination of psychosocial factors in pSLE patients.
Main Results:
- Most pSLE patients develop damage within 5-10 years, affecting musculoskeletal, ocular, renal, and neuropsychiatric systems.
- Improved management leads to longer survival but increased cumulative disease damage.
- Premature atherosclerosis and osteoporosis are significant, prevalent morbidities in pSLE, increasing risks for cardiovascular events and fractures.
Conclusions:
- pSLE is an incurable, severe disease impacting children during critical developmental stages.
- Increased longevity in pSLE patients results in higher rates of premature atherosclerosis and osteoporosis.
- Further large-scale, long-term studies are essential for improved pSLE prognosis and tailored treatment development.
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.
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