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A Clinical Overview of Systemic Lupus Erythematosus in Childhood

Bianca A. Lang MD1
Earl D. Silverman MD2
 1 Assistant Professor of Pediatrics, Department of Pediatrics, The Izaak Walton Killam Children's Hospital, Dalhousie University, Halifax, Nova Scotia, Canada
2 Associate Professor of Pediatrics and Immunology, Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada

Systemic lupus erythematosus (SLE) is an autoimmune disorder resulting in inflammation of multiple organ systems. Although SLE is not common in children, it must be considered in the differential diagnosis of any child who has multisystem disease. The onset of SLE in children may be acute, with severe disease affecting one or more major organ systems, or it may be insidious, with intermittent symptoms of fever, malaise, rashes, arthritis, or pleuritis. SLE also must be considered if a child is found to have a positive antinuclear antibody (ANA) test result. Although almost all children who have SLE have a positive result, this laboratory test alone does not establish the diagnosis of SLE.

Classification Criteria

Eleven disease manifestations are included in the revised classification criteria for SLE established by the American College of Rheumatology (Table 1). These criteria were established as a classification tool to guide in selecting patients for clinical and laboratory studies rather than as a diagnostic system. Nevertheless, they have proven useful in the assessment of both pediatric and adult patients who may or may not have SLE.

Epidemiology

Childhood onset of SLE accounts for approximately 20% of all cases. The annual incidence in the United States is approximately 0.6 cases per 100 000 total population, with higher rates found in the African-American, Oriental, and Hispanic populations.




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