Pediatric emergency medicine trisk 2931 2931
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but when seen are characteristic ( Fig. 101.1 ). Evidence of renal disease is present in
approximately 50% of children with SLE at the time of presentation, with nearly 90%
developing some degree of renal involvement during the course of their disease. This is
significantly higher than in adult patients, in whom renal disease develops in about
half. Lupus nephritis is usually asymptomatic, although close questioning often reveals
nocturia due to impaired renal concentrating mechanisms. Edema or hypertension may
be clues to involvement of the kidney. Despite significant improvements in treatment,
the extent of renal involvement remains the single most important determinant of
prognosis in SLE, and therefore will highly influence choice of immunosuppressive
therapy. Thus, most children with evidence of kidney disease undergo renal biopsy to
more precisely characterize the pathology and help optimize the therapeutic regimen.
Clinical evidence of CNS involvement may occur at disease onset or later in the
course. Symptoms and signs referable to the CNS include headache, seizures,
polyneuropathy, hemiparesis/hemiplegia, and ophthalmoplegia. Particularly in the ED
setting, the clinician should be aware of the risk of stroke (both thrombotic and
hemorrhagic) and of sinus vein thrombosis. Chorea is the most common movement
disorder and may be a presenting sign; Lyme disease (LD) and rheumatic fever must
also be considered in such cases. Cranial nerve palsies most commonly involve the
optic nerve, trigeminal nerve, and nerves controlling the extraocular muscles.
Myasthenia gravis should be excluded if any extraocular muscles are involved.
Neuropsychiatric manifestations include mood disorders, hallucinations, memory
alterations, and psychosis; rarely, psychiatric symptoms may be the first clinical
manifestation of childhood lupus.
Pericarditis is the most prevalent form of cardiopulmonary involvement in SLE.
Myocarditis occurs less frequently. Heart murmurs caused by valvular lesions are not
common, but asymptomatic vegetations on valve leaflets are seen at autopsy in most
patients (Libman–Sacks endocarditis), which is why patients with SLE are at increased
risk for subacute bacterial endocarditis. Myocardial infarctions have been reported in
children with lupus, and the possibility of myocardial ischemia should be kept in mind
if a child with lupus develops acute chest pain.
Pleuropulmonary involvement occurs in more than 50% of cases of SLE. Unilateral
or bilateral pleural effusions may occur, and pulmonary hemorrhage, although
uncommon, also occurs in children with SLE. Pulmonary function testing (PFT)
demonstrating an elevated DLCO offers a readily available, noninvasive technique for
identifying blood in the lungs. Pulmonary embolus, particularly in children with
antiphospholipid antibodies, also must be considered in children with the acute onset of
chest pain. For any SLE patient with pleuropulmonary manifestations, disease-related
involvement must be distinguished from intercurrent infection, CHF, aspiration
pneumonia, and renal failure.
Common GI manifestations include nausea, vomiting, and anorexia. Persistent
localized abdominal pain should suggest specific organ involvement, such as
