What is the best course of action for an 11-year-old patient with a 4-year history of Systemic Lupus Erythematosus (SLE) presenting with occasional headache and vomiting, with normal Complete Blood Count (CBC), urinalysis, creatinine, Serum Glutamic-Pyruvic Transaminase (SGPT), and Serum Glutamic-Oxaloacetic Transaminase (SGOT) and no signs of a lupus flare?

Neuropsychiatric Evaluation for CNS Lupus

This 11-year-old with lupus presenting with headache and vomiting requires immediate neuroimaging and comprehensive neuropsychiatric assessment to exclude CNS lupus, even with normal laboratory parameters and no systemic flare.

Immediate Diagnostic Workup Required

Neuroimaging is Essential

• Brain MRI with contrast should be obtained urgently to evaluate for CNS involvement, including posterior reversible encephalopathy syndrome (PRES), vasculitis, or other structural abnormalities 1, 2
• PRES can occur even in normotensive patients with SLE and presents with headache, vomiting, and seizures 2
• MRI findings of vasogenic edema in posterior brain regions would confirm PRES, which requires immediate immunosuppressive therapy 2

Additional Laboratory Assessment

• Obtain inflammatory markers (ESR and CRP) to distinguish between lupus activity and infection 3
  • CRP >50 mg/L strongly suggests superimposed infection rather than lupus flare 1, 3
  • Normal CRP with elevated ESR may indicate lupus activity 3
• Check complement levels (C3, C4) and anti-dsDNA antibodies as primary serological markers for disease activity 3
• Assess for antiphospholipid antibodies given their association with neuropsychiatric manifestations 1

Clinical Context and Differential

Neuropsychiatric Lupus Considerations

• Neurological involvement occurs frequently in SLE, with headache being one of the most common syndromes 1
• "Lupus headache" as defined by SLEDAI-2K is rare (only 1.5% of patients) and should only be diagnosed after excluding other causes 4
• Primary headaches (tension-type and migraine) are actually more common in lupus patients (pooled prevalence 26.8%) and are associated with depression rather than disease activity 4
• The combination of headache with vomiting raises concern for increased intracranial pressure or CNS vasculitis 5

Critical Pitfall to Avoid

• Do not assume headache is benign simply because routine labs are normal 1, 4
• Normal CBC, creatinine, and liver enzymes do not exclude active CNS lupus 1
• Neuropsychiatric manifestations can occur independently of systemic disease activity 4

Management Algorithm

If Neuroimaging Shows Abnormalities

• Immediate consultation with pediatric rheumatology and neurology 6
• Consider pulse methylprednisolone (typically 15-30 mg/kg/day for 3 days) followed by high-dose oral corticosteroids 2
• Monthly cyclophosphamide may be required for severe CNS involvement 2
• Ensure patient is on hydroxychloroquine, as it reduces disease flares including neuropsychiatric manifestations 1, 4

If Neuroimaging is Normal

• Evaluate for primary headache disorders (tension-type or migraine) using ICD-II criteria 5
• Screen for depression and assess quality of life, as primary headaches in lupus correlate with these factors rather than disease activity 4
• Consider empiric treatment for migraine or tension headache if criteria are met 5
• Hydroxychloroquine may reduce frequency of primary headaches 4

Infection Must Be Excluded

• Obtain blood cultures if fever develops 1
• Consider lumbar puncture if meningitis or encephalitis is suspected, particularly if immunosuppressed 1
• Test for cytomegalovirus (CMV) if on high-dose corticosteroids or pulse therapy, as CMV can mimic active SLE 1

Monitoring and Follow-up

• Close monitoring is essential given the patient's young age and 4-year disease duration 1
• Younger age at disease onset is a consistently reported risk factor for higher disease flare rates 1
• If symptoms persist or worsen despite treatment, repeat neuroimaging and consider lumbar puncture 1
• Document cognitive function (memory, attention, concentration, word-finding) at baseline and follow-up visits 1