Systemic Lupus Erythematosus(SLE) in childhood shares all of the clinical features observed in adults with SLE but with varying frequencies and unique consequences. Until the last
two decades, survival in pediatric SLE was poor, primarily because of the increased frequency of kidney and neurological manifestations. Recent advances in diagnosis and therapy have paralleled those achieved in adult SLE. However, it is important not to think of disease management in children as treating “little adults”. Because of the variable effects of SLE and its therapy on both physical and emotional growth, it is important to develop a logitudinal plan that will ease the transition of these consequences through adolescent into adulthood. Approximately one fourth of all systemic lupus cases start during childhood.

Most of these occur after age five, with peak incidence in late childhood and adolescence. As in adult SLE, females are more often diagnosed than males, particularly after puberty. It appears that female sexual maturation may be a factor in childhood lupus pathogenesis. Interestingly, there is a high incidence of lupus in males with Klinefelter’s syndrome, a genetic condition where an individual of male appearance has an extra female, or X, chromosome. Genetic factors play a major role in the predisposition for developing lupus. A child with lupus is more likely to have a family member with SLE or other connective tissue disease. One study has demonstrated a high prevalence of anti-nuclear antibodies in the asymptomatic parents or siblings of pediatric lupus patients. Keep in mind, however, that like adults, children may have an anti-nuclear antibody test that is “false-positive” that is, without associated lupus or related collagen vascular disease.

The clinical manifestations of childhood lupus often begin insidiously. Vague symptoms such as joint pain, fatigue, weight loss, fevers and rash may persist for months before an accurate diagnosis is made. Whereas, lupus symptoms may be relatively mild or “incomplete” in some adults, most children with SLE will either present with or develop severe systemic features. Children appear to have a higher incidence of anemia, spleen enlargement, and kidney involvement. The higher utilization of corticosteroids to control these features in children can have negative effects on bone growth and density (leading to osteoporosis,) bone blood flow (leading to ost eone c ros i s )   and  resistance to infection. Immunosupressive or steroid-sparing drug regimens may also raise the risk for infection.

Preventative strategies to control these complications are crucial. These include dietary measures, calcium supplements and appropriate timing of immunizations. Another complication of childhood lupus that is recognized with increased frequency is the early occurrence of atherosclerotis. Careful attention must be paid even in childhood to assess and regulate blood pressure and cholesterol with diet and, if necessary, medication. Health care providers and family of a child with lupus must comprehend the physical and emotional impact of disease and its treatment on his or her development. As survival for childhood lupus has improved in the last decade, these children are more likely to have functional limitations or disabilities that span into young adulthood. Whether the limitations become a handicap depends on how a child‘s parents, family, peers and community respond to that individual. Unlike other diseases, systemic lupus may produce less visible disabling features such as fatigue or cognitive difficulties. These, along with the uncertainty of prognosis, steroid-related changes in body image and health-care related absenteeism, can produce social isolation and delayed psychosocial or vocational development.

Healthcare providers and parents need to facilitate a child’s transition over these barriers. Dr. Patience White from the Children’s National Medical Center and others involved in transition research have developed principles for “success on the road to adulthood”. An appropriate transition plan is one which begins on the day of diagnosis and fosters a trend from parental-advocacy in early childhood to self-advocacy in adolescence. Successful transition from the child with lupus is accomplished when he or she enters their independent living.