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corticosteroids, immunosuppressive agents, and/or plasmapheresis. Treatment of GI
hemorrhage is described in the “Gastrointestinal Complications” section.
Although less common than in systemic juvenile idiopathic arthritis (sJIA),
disseminated intravascular coagulation (DIC) associated with macrophage activation
syndrome (MAS) may occur in SLE, with or without an associated infection.
Therefore, patients with thrombocytopenia and severe bleeding should be investigated
with prothrombin time, partial thromboplastin time (PTT), fibrin split products, ferritin,
and examination of the peripheral smear. Lupus also appears to predispose to a
particularly malignant form of thrombotic thrombocytopenic purpura. Mortality rates
are high, despite general support in ICUs and aggressive treatment with pheresis and
immunosuppression. Outcomes are optimal when the diagnosis is suspected early and
treatment is initiated rapidly.
The presence of a circulating lupus anticoagulant does not lead to a bleeding
diathesis unless associated with significant thrombocytopenia; on the contrary, these
patients are at increased risk of deep venous or arterial thrombosis. Prolongation of
PTT and chronic false-positive serologic tests for syphilis are the usual clues to the
presence of these autoantibodies. Antiphospholipid antibodies may also be measured,
and the antiphospholipid antibody syndrome is associated with significant morbidity
and mortality. Significant thrombosis or pulmonary embolus in a child with SLE is an
indication for immediate anticoagulation.
Neurologic Complications. Seizures (see Chapter 72 Seizures ) and altered states of
consciousness (see Chapters 17 Coma and 97 Neurologic Emergencies ) are the most
common manifestations of CNS involvement in SLE. Other possible causes of seizures
in patients with SLE include hypertension, infection (meningitis, encephalitis, or
abscess), and uremia. Coma is not a primary manifestation of SLE but may result from
meningitis or CNS hemorrhage related to thrombocytopenia. Therefore, patients with
SLE who develop seizures or altered states of consciousness require urgent imaging,
specifically a computed tomography (CT) scan, with and without contrast. Magnetic
resonance imaging (MRI) may be required because the differential diagnosis includes
lupus cerebritis. Patients should have repeated assessments with special attention to
blood pressure and neurologic findings, as well as the following investigations: CBC,

PT/PTT, electrolytes, BUN, creatinine, and urinalysis. Once space-occupying lesions
have been excluded, lumbar puncture (including measurement of opening pressure)
should be performed, with CSF sent for routine studies as well as special stains to look
for opportunistic organisms such as fungi and acid-fast bacilli.
If CNS manifestations are believed to be caused by active vasculitis, IV
corticosteroid therapy should be initiated. In the presence of deteriorating mental
function, “pulse” methylprednisolone (30 mg/kg, 1.5 g maximum), IV
cyclophosphamide, or plasmapheresis may be beneficial.