Pediatric emergency medicine trisk 2930 2930
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Although SLE is often considered a disease of adulthood, up to 20% of lupus patients
are diagnosed during the first two decades of life. Childhood SLE affects girls more
often than boys but this gender difference occurs to a lesser extent than in adults.
Incidence and prevalence rates vary by ethnicity and are higher in Hispanic, Asian,
Native American, and African populations. The mean age of diagnosis in children is
approximately 12 to 13 years. The onset of SLE may be insidious or acute. The initial
presentation usually includes constitutional features, such as fever, malaise, and weight
loss, in addition to manifestations of specific organ involvement such as rash,
pericarditis, arthritis, or seizures. Because virtually any part of the body may be
affected by SLE, patients may present with a bewildering variety of signs and
symptoms. Although many of these are nonspecific, the examiner’s level of suspicion
for possible SLE should increase as the number of involved organ systems increases.
Further, although SLE presents with a wide array of symptoms, the majority of
pediatric cases present with a recognizable constellation of complaints related to
musculoskeletal, cutaneous, renal, and hematologic involvement. In French and
Canadian studies, the most common presenting manifestations in children are
hematologic (anemia, lymphopenia, leukopenia, and/or thrombocytopenia);
mucocutaneous (malar rash and/or ulcers); musculoskeletal (arthritis or arthralgia);
presence of fever; and renal abnormalities (nephritis or nephritic syndrome). (Please
refer to the SLICC criteria discussed above for specific details about making the
diagnosis.)
Triage Considerations
Fever in a child with SLE represents a potential emergency. Children with SLE are at
increased risk of infections from their disease activity and also from the
immunosuppressive therapies that they receive to control their illness. Patients with
fever should be evaluated rapidly and thoroughly, and often require empiric broadspectrum antibiotics while awaiting the results of the diagnostic evaluation. Patients
taking corticosteroids may require stress doses during acute febrile illness. Children
with SLE are also at increased risk for a wide variety of cardiac, pulmonary, and
gastrointestinal (GI) complications, many of which are life-threatening. See Table
101.3 , which describes the complications of SLE.
Clinical Assessment
Arthritis in SLE is usually symmetric, involving both large and small joints. Swollen
joints may be quite painful, but they are usually not erythematous. Cutaneous lesions
are present in more than 85% of patients with SLE. The typical malar rash with
butterfly distribution is present at diagnosis about half the time. Features that help to
distinguish it from other rashes are sparing of the nasolabial folds, extension onto the
nose, and extension of the rash onto the chin. Painless oral or nasal ulcerations,
alopecia, and photosensitivity are common. Discoid lesions are less frequent in children
