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What's new in paediatric SLE?
Emily von Scheven1, Aysin Bakkaloglu
1Pediatric Rheumatology, University of California, San Francisco, CA 94143, USA. evonsche@peds.ucsf.edu
Insights
Childhood-onset systemic lupus erythematosus (SLE) is severe and can lead to early atherosclerosis. Improved treatments are needed to prevent cardiovascular complications in these young patients.
Area of Science:
- Pediatric Rheumatology
- Cardiovascular Disease in Autoimmunity
Background:
- Systemic lupus erythematosus (SLE) affects 15-20% of children under 16, often presenting with greater severity and kidney involvement.
- While childhood SLE mortality has decreased due to earlier diagnosis and better care, long-term complications are emerging.
- Treatment protocols for childhood lupus nephritis are primarily based on adult data, lacking specific pediatric clinical trial evidence.
Purpose of the Study:
- To review the challenges and advancements in managing childhood-onset SLE.
- To highlight the significant risk of early-onset atherosclerosis and myocardial ischemia in young SLE patients.
- To emphasize the need for pediatric-specific research on SLE pathogenesis and preventative strategies.
Main Methods:
- Review of current literature on childhood-onset SLE.
- Analysis of treatment strategies adapted from adult SLE management.
- Examination of secondary complications, particularly atherosclerosis, in pediatric SLE cohorts.
Main Results:
- Childhood SLE frequently involves critical organs and carries a higher risk of severity.
- Improved survival has revealed increased incidence of early atherosclerosis and myocardial ischemia in young adults with childhood-onset SLE.
- Disease and treatment factors contribute to atherogenesis in SLE patients.
Conclusions:
- There is a critical need for pediatric-specific clinical trials to establish treatment efficacy and safety in childhood SLE.
- Preventative strategies against atherosclerosis are crucial for improving long-term outcomes in individuals with childhood-onset SLE.
- Further research into the pathogenesis of SLE and its cardiovascular complications in children is essential.
Abstract:
Although more commonly presenting in adulthood, approximately 15-20% of systemic lupus erythematosus (SLE) cases occur before age 16 years. Unfortunately, SLE is usually more severe when presenting in childhood, and frequently involves vital organs such as the kidney. Over the past several decades, mortality rates have dropped, largely due to earlier diagnosis, improved management of the SLE and improved general medical care to reduce infection. Treatment strategies for nephritis in children is largely adopted from experience in adults, and the recent advances in therapeutic options for adults have brought new treatment to children. However, determining efficacy is difficult due to the absence of clinical trial data. Furthermore, determination of safety in a developing child or adolescent cannot be extrapolated from adult studies. As survival has improved, numerous secondary complications have emerged, including early atherosclerosis. As for adults with SLE, it is generally accepted that atherogenesis in SLE results from both disease- and treatment-related factors. Most surprising is that persons with childhood-onset SLE can develop myocardial ischaemia as early as 20-30 years of age. Better understanding of the pathogenesis and development of preventative strategies is needed to ensure that these young people do not succumb to atherosclerosis instead of to SLE.
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