A clinical overview of systemic lupus erythematosus in childhood.

Abstract

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.