Pediatric emergency medicine trisk 733

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


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


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


pancreatitis or gastric ulcer, both of which may occur from the disease or secondary to
medical therapy. Malabsorption syndrome may be a manifestation of SLE. When
accompanied by melena, it suggests poorly controlled disease complicated by GI
vasculitis. This is associated with a 50% mortality rate without expeditious evaluation
and treatment. Of course, abdominal pain in SLE is not always related to the
underlying disease but may stem from other causes, including appendicitis, ruptured
ovarian cyst, or pelvic inflammatory disease. Further complicating evaluation is the
fact that manifestations of any of these conditions may be masked or altered by the
corticosteroids and immunosuppressive agents most patients receive.
Mild to moderate anemia is common in SLE. Hemolytic anemia associated with a
positive Coombs test is most characteristic. An acute decrease in the hemoglobin or
hematocrit should alert the physician to the possibility of internal hemorrhage or

massive hemolysis. Autoimmune thrombocytopenia, even in the absence of offending
drugs, is commonly seen in SLE; up to 20% of adults initially diagnosed with
idiopathic thrombocytopenic purpura (ITP) progress to full-blown lupus over the
ensuing years. Leukopenia and lymphopenia are additional hematologic abnormalities
characteristically seen in SLE; apart from viral infections and drug toxicity, few other
conditions cause children’s lymphocyte counts to fall to <1,000/mm3. Circulating
antibodies to specific clotting factors, deficiencies of one or more clotting factors, and
abnormal platelet function, often lead to abnormal hemostasis in SLE. A specific
circulating anticoagulant, the “lupus anticoagulant,” has been described in up to 10% of
patients. The antibody is so named because in vitro assays of coagulation are prolonged
in its presence. In vivo, this antibody predisposes to arterial or venous thrombosis.
Proteinuria, hematuria, and cellular casts are the usual urinary abnormalities. Acute
renal failure and nephrotic syndrome are possible complications of SLE (see Chapter
100 Renal and Electrolyte Emergencies ).
The most important single test in children suspected of having SLE is measurement
of antinuclear antibody (ANA) titers. Up to 2% of normal children have low to
intermediate titers of ANA; in most cases, these antibodies are transient by-products of
a viral infection. In SLE, the ANA titer is typically quite high—significant levels are
greater than 1:640 and often it is accompanied by antibodies to double-stranded DNA,
a more specific marker for lupus. The level for the anti–double-stranded DNA (anti-ds
DNA) should be above the laboratory reference range, and if tested by ELISA, should
be twice that of the upper limit of the laboratory reference range. Antiphospholipid
antibodies may be positive as determined by detecting any of the following: the lupus
anticoagulant, false-positive RPR, medium- or high-titer anticardiolipin (IgA, IgG, or
IgM), or presence of anti–beta-2 glycoprotein I antibodies (IgA, IgG, or IgM). Finally,
low complement levels: C3, C4, or total CH50, and a direct Coombs test in the absence
of hemolytic anemia, are the additional immunologic tests that may point the clinician
to a diagnosis of SLE. Nonetheless, it must be remembered that SLE may only be