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Pediatric SLE—towards a comprehensive management plan

Nature Reviews Rheumatology volume 7, pages 225–233 (2011)Cite this article

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Abstract

Systemic lupus erythematosus (SLE) results from complex abnormalities of the innate and acquired immune systems. For reasons that are currently not well understood, the disease course and phenotype associated with SLE, although quite variable, are generally more severe when the diagnosis is made during childhood. Active disease, infections, lupus nephritis, and neuropsychiatric SLE manifestations are associated with higher morbidity and mortality. Unlike in adult-onset SLE, systemic glucocorticoid therapy and immunosuppressive medications are needed for the treatment of the majority of children and adolescents with SLE. The complex nature of childhood-onset SLE demands a comprehensive, multidisciplinary management approach that considers the patients' growth and development, their educational needs, and the unpredictable course of SLE and its complications.

Key Points

  • Disease severity and issues of growth, development, educational and psychosocial needs are some of the features that distinguish pediatric SLE (pSLE) from adult-onset SLE (aSLE)

  • Disease indices used to measure disease activity and damage in aSLE have been validated and are used in pSLE

  • Patients with pSLE require monitoring for adherence and response to treatment, occurrence of disease flares and damage, as well as health-maintenance issues such as immunizations, bone density and premature atherosclerotic disease

  • Patients with pSLE are at a high risk of tissue damage, and require prompt, often aggressive, management

  • Research is ongoing to improve the available tests for assessing disease activity in pSLE, especially lupus nephritis

  • Treatments for pSLE currently have similar limitations as those for aSLE; however, improvement in our understanding of the B-cell biology of SLE may result in more therapeutic options for pSLE

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Figure 1: Effects of glucocorticoid therapy on gene expression profiles in patients with SLE.

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Acknowledgements

This work is supported by NIH grants 5U01-AR51868 and P60-AR047884.

C. P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. William S. Rowe Division of Rheumatology & Lupus Center, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, 3333 Burnet Avenue, Cincinnati, 45229-3039, OH, USA

    Hermine I. Brunner & Jennifer Huggins

  2. Division of Pediatric Rheumatology, Children's Memorial Hospital, Northwestern University Feinberg School of Medicine, 2300 Children's Plaza, Chicago, 60614-3363, IL, USA

    Marisa S. Klein-Gitelman

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H. I. Brunner and J. Huggins researched data for the article. H. I. Brunner and M. S. Klein-Gitelman made substantial contributions to discussing the content of the article. All authors took part in writing, reviewing and editing the manuscript before submission.

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Correspondence to Hermine I. Brunner.

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Brunner, H., Huggins, J. & Klein-Gitelman, M. Pediatric SLE—towards a comprehensive management plan. Nat Rev Rheumatol 7, 225–233 (2011). https://doi.org/10.1038/nrrheum.2011.15

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