Pediatric emergency medicine trisk 2930 2930

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