Systemic Lupus Erythematosus in Children and Adolescents

Abstract

1. Beth S. Gottlieb, MD, MS* 2. Norman T. Ilowite, MD† 1. *Assistant Professor of Pediatrics, Albert Einstein College of Medicine; Chief, Division of Rheumatology, Schneider Children's Hospital, Hyde Park, NY 2. †Professor of Pediatrics, Albert Einstein College of Medicine; Chief, Division of Rheumatology, Children's Hospital at Montefiore, Bronx, NY After completing this article, readers should be able to: 1. List the most common presenting symptoms of systemic lupus erythematosus (SLE) in the pediatric population. 2. Describe the patient who is most likely to have secondary Raynaud phenomenon. 3. Recognize why antinuclear antibody testing lacks specificity. 4. Know the specific serologic markers for SLE. 5. Discuss the treatment for SLE. Systemic lupus erythematosus (SLE) is a multisystem, chronic but often episodic, autoimmune disease that is characterized by the presence of antinuclear antibodies (ANA). Because SLE can present with a number of signs and symptoms, the diagnosis often is considered in children who have prolonged unexplained complaints. Approximately 20% of all patients who have SLE are diagnosed in childhood. The onset of SLE is rare in those younger than 5 years of age; most pediatric patients are diagnosed in adolescence. SLE is considered a predominantly female disease, and although most affected patients are female, the ratio changes with age. Prior to puberty, the female-to-male ratio is 3:1; after puberty, the ratio becomes 9:1. Aside from this sex difference, there also are marked racial differences observed in SLE. Native Americans are most susceptible to developing SLE, followed by African-Americans, Hispanics, Chinese, and Filipinos. The disease tends to be more severe in African-Americans and Hispanics. SLE is a multigenic disease. A patient who has SLE is more likely to have a relative who has either SLE or another autoimmune disease such as thyroiditis or insulin-dependent diabetes. Epidemiologic, twin, and human leukocyte antigen data suggest a strong genetic contribution to the etiology of SLE, but the exact cause is unknown. Multiple factors confer risk, including abnormalities in the metabolism of sex hormones, particular foods that have been found to be immunostimulatory in animals, and infectious agents. Overall, it has been suggested that an environmental trigger is necessary in a …