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having SLE. The proposed scoring system is currently being studied to see in what way
the sensitivity and specificity compares to the ACR and SLICC classification systems.
More research is needed to determine if the EULAR/ACR classification system is
applicable for diagnosing SLE in children. It is probable that in the future, this new
classification will replace the 1997 ACR and 2012 SLICC classification systems.

FIGURE 101.2 Mucosal lesions (macules and ulcers) of the palate in an adolescent girl with active
lupus.

Goals of Treatment
SLE is often more severe in children than in adults. Although adult lupus patients are
more likely to die of complications, children and adolescents with lupus are more likely
to succumb earlier, during the acute stages of the disease. Common causes of death
within the first 2 years of diagnosis are pancreatitis, pulmonary hemorrhage, infection,
thromboembolic disease, and active neuropsychiatric disease. Delayed diagnosis and
treatment are strong risk factors for morbidity and mortality in pediatric lupus. In view
of the fact that cumulative disease activity over time correlates with damage from the
disease, expedient diagnosis and appropriately aggressive treatment is particularly
critical for children. Thus, pediatricians need to maintain a high index of suspicion for
lupus, and physicians experienced in the care of children with SLE should participate
in the diagnosis and management of all pediatric lupus patients.

Clinical Considerations
Clinical Recognition