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Therapy Insight: cardiovascular disease in pediatric systemic lupus erythematosus

Abstract

In 15–20% of cases, systemic lupus erythematosus (SLE) presents before the age of 18 years, and such early-onset SLE seems to be particularly severe. SLE is an independent risk factor for premature atherosclerosis and death in young, premenopausal women with SLE, even after controlling for traditional cardiovascular risk factors. Children and adolescents with SLE are particularly susceptible to this long-term threat to their cardiovascular health because they have an increased disease severity and a lengthy disease burden. Factors that contribute to premature atherosclerosis include the inflammatory and immune abnormalities that are intrinsic to SLE, primary dyslipidemias, and the secondary effects of treatments such as corticosteroids. However, few rheumatologists provide appropriate preventive or management strategies for the increased atherosclerosis risk in this age-group. Screening should be performed on a regular basis, including evaluation of, and counseling for, traditional risk factors. Studies of treatment in pediatric patients are limited, and treatment strategies are often extrapolated from adult studies. Statins hold promise because they have both lipid-lowering and anti-inflammatory effects. There have been few studies of the use of statins in adults or adolescents with SLE; however, trials are currently underway to address the safety and efficacy of statin use in pediatric SLE.

Key Points

  • Premature atherosclerosis is a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE)

  • A diagnosis of SLE is an independent contributor to the risk of atherosclerosis in addition to traditional cardiovascular risk factors

  • Inflammatory changes characteristic of SLE are likely to contribute to premature atherosclerosis

  • Strategies to decrease the risk of atherosclerosis should be initiated in adolescents and young adults in whom SLE developed during childhood or adolescence

  • Dietary and lifestyle changes are recommended for all children and adolescents with SLE, and lipid-lowering agents should be considered in individuals at high risk of cardiovascular events

  • Whether adolescents and young adults with SLE should be treated aggressively with statins or other agents is unknown; appropriate studies are currently underway

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Figure 1: Summary of the multiple factors that are known to contribute to abnormal lipid profiles in SLE.
Figure 2: Immunologic effects of statins.

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Acknowledgements

SP Ardoin would like to acknowledge support from the American College of Rheumatology Research and Education Foundation. C Sandborg and L Schanberg would like to acknowledge grant support from the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS NO1 AR22256 and NIAMS-090).

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Correspondence to Christy Sandborg.

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Sandborg, C., Ardoin, S. & Schanberg, L. Therapy Insight: cardiovascular disease in pediatric systemic lupus erythematosus. Nat Rev Rheumatol 4, 258–265 (2008). https://doi.org/10.1038/ncprheum0789

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