What is the best approach to manage dizziness and vomiting in an 11-year-old patient with a 4-year history of Systemic Lupus Erythematosus (SLE), no current active flare, normal laboratory tests including Complete Blood Count (CBC), urinalysis, Serum Glutamic-Pyruvic Transaminase (SGPT), Serum Glutamic-Oxaloacetic Transaminase (SGOT), Complement 3 (C3), and creatinine, and no seizures in the past 6 months, with symptoms occurring upon waking or after physical activity?

Evaluation and Management of Dizziness and Vomiting in Pediatric SLE

Immediate Diagnostic Priority: Rule Out Neuropsychiatric Lupus (NPSLE)

This child requires urgent evaluation for neuropsychiatric lupus erythematosus (NPSLE), despite normal laboratory markers, because neuropsychiatric manifestations can occur independently of systemic disease activity and represent potentially serious CNS involvement. 1

Critical First Steps in Diagnostic Workup

The diagnostic evaluation must mirror what would be done for any child presenting with these symptoms, while maintaining high suspicion for NPSLE 1:

• Obtain brain MRI with contrast to identify structural lesions, white matter abnormalities (present in 50-55% of NPSLE seizure cases), cerebral atrophy, or ischemic changes that could explain symptoms 1

• Perform EEG to exclude subclinical seizure activity, as seizures can manifest atypically and EEG abnormalities occur in 60-70% of SLE patients with seizure disorders 1

• Check antiphospholipid antibodies (anticardiolipin, anti-β2-glycoprotein I, lupus anticoagulant) if not previously done, as these are associated with neuropsychiatric manifestations and thrombotic/ischemic events 1

• Lumbar puncture with CSF analysis should be strongly considered to exclude CNS infection (especially in patients on any immunosuppression) and may reveal inflammatory markers suggestive of NPSLE 1

Attribution of Symptoms to NPSLE

The diagnosis requires a comprehensive, multidisciplinary approach considering 1:

• Timing and type of manifestation - dizziness and vomiting upon waking or after physical activity could represent vestibular involvement, posterior circulation ischemia, or autonomic dysfunction
• Presence of risk factors - even with normal systemic markers, NPSLE can occur; positive antiphospholipid antibodies would significantly increase suspicion 1
• Abnormal neuroimaging or CSF findings - these would favor NPSLE attribution 1
• Exclusion of mimics - infections, metabolic disturbances, medication effects, and other causes must be systematically ruled out 1

Differential Diagnosis Beyond NPSLE

Non-Lupus Causes to Exclude

• Orthostatic hypotension - check orthostatic vital signs, particularly if on any medications affecting blood pressure 2

• Vestibular disorders - benign paroxysmal positional vertigo or vestibular neuritis can cause positional dizziness and vomiting

• Migraine-associated vertigo - common in this age group and can mimic NPSLE

• Medication effects - review all current medications including hydroxychloroquine (though vestibular toxicity is rare at appropriate dosing) 2

• Metabolic/endocrine causes - despite normal basic labs, consider glucose monitoring, thyroid function, and electrolytes during symptomatic episodes

Treatment Algorithm Based on Findings

If NPSLE is Confirmed (Inflammatory Mechanism)

Initiate combination therapy with glucocorticoids and immunosuppressive agents 1:

• Pulse intravenous methylprednisolone (typically 15-30 mg/kg/day, maximum 1 gram daily for 3 days) for acute management 1

• Followed by oral prednisone (1-2 mg/kg/day) with plan for gradual taper based on response 1

• Add cyclophosphamide for severe, refractory NPSLE - the combination of pulse IV methylprednisolone and IV cyclophosphamide showed 95% response rate (18/19 patients) versus 54% with methylprednisolone alone in the only RCT of severe NPSLE 1

• Consider mycophenolate mofetil as an alternative immunosuppressant, though it has not shown efficacy specifically in neuropsychiatric disease 1

If Antiphospholipid Antibodies are Positive

• Initiate antiplatelet therapy with low-dose aspirin (81 mg daily) for primary thrombosis prevention 1, 2

• Consider anticoagulation if there is evidence of thrombotic/ischemic mechanism on imaging, even without prior thrombotic events 1

• Combination therapy (immunosuppression plus anticoagulation) may be necessary if both inflammatory and thrombotic mechanisms coexist 1

If Evaluation is Negative for NPSLE

• Optimize hydroxychloroquine dosing (not exceeding 5 mg/kg actual body weight) as it reduces overall disease activity and should be continued 1, 2

• Treat symptomatically based on identified cause (vestibular rehabilitation, antimigraine therapy, etc.)

• Maintain close monitoring with repeat evaluation every 3 months given the episodic nature of symptoms 1, 2

Critical Monitoring Parameters

• Serial neurological examinations to detect any progression or new focal findings 1, 2

• Disease activity monitoring every 3-6 months including CBC, creatinine, urinalysis, C3/C4, and anti-dsDNA even when clinically stable 1, 2

• Ophthalmologic screening annually if on hydroxychloroquine (baseline already established after 4 years of treatment) 2, 3

Important Clinical Pitfalls to Avoid

• Do not assume normal systemic markers exclude NPSLE - neuropsychiatric manifestations can occur independently of extraneural disease activity, and no single test definitively diagnoses or excludes NPSLE 1

• Do not delay neuroimaging - structural lesions require identification before attributing symptoms to functional causes 1

• Do not empirically escalate immunosuppression without comprehensive workup excluding infection and other mimics 2

• Do not discontinue hydroxychloroquine - it should be maintained unless specifically contraindicated, as it reduces mortality and disease activity 1, 2

• Avoid prolonged high-dose glucocorticoids - if immunosuppression is needed, add steroid-sparing agents early to facilitate tapering below 7.5 mg/day 1, 2