In an 11‑year‑old with known systemic lupus erythematosus presenting with recurrent nausea and vomiting on waking, after large meals, or after exercise and no progressive headache, what is the appropriate diagnostic work‑up and management?

Diagnostic Work-Up and Management

Initial Clinical Assessment

This 11-year-old with lupus presenting with episodic nausea and vomiting triggered by waking, heavy meals, or exercise—without progressive headaches—requires urgent evaluation to exclude neuropsychiatric lupus erythematosus (NPSLE), particularly cranial neuropathy or CNS inflammation, before considering cyclic vomiting syndrome as an alternative diagnosis. 1

Key Diagnostic Priorities

The diagnostic approach must first rule out NPSLE manifestations, as neuropsychiatric symptoms can occur independently of systemic lupus activity and carry significant morbidity if untreated 1:

• Brain MRI with contrast is mandatory using T1/T2, FLAIR, diffusion-weighted imaging, and gadolinium-enhanced sequences to identify structural lesions, white matter abnormalities, cerebral atrophy, ischemic changes, or brainstem involvement (abnormalities present in 50-55% of NPSLE cases) 2, 1, 3

• EEG should be performed to exclude subclinical seizure activity, as seizures manifest atypically in pediatric SLE and EEG abnormalities occur in 60-70% of SLE patients with seizure disorders 1, 4

• Lumbar puncture with CSF analysis is essential to exclude CNS infection, particularly in immunosuppressed patients 2, 4

• Antiphospholipid antibodies (anticardiolipin, anti-β2-glycoprotein I, lupus anticoagulant) must be checked if not previously done, as these associate with neuropsychiatric manifestations and thrombotic/ischemic events 1, 3

Common Pitfall to Avoid

Do not attribute gastrointestinal symptoms solely to benign causes without neuroimaging in pediatric lupus patients. Cranial neuropathy affecting the eighth nerve occurs in 1-5% of SLE patients and can present with nausea and vomiting 3. Additionally, NPSLE commonly occurs within the first year after SLE diagnosis (50-60% of cases) and often coincides with generalized disease activity (40-50%) 2.

If NPSLE is Confirmed

Acute Immunosuppressive Therapy

• Pulse intravenous methylprednisolone (15-30 mg/kg/day for 3 days) followed by oral prednisone (1-2 mg/kg/day) with gradual taper based on response 1, 3

• Add intravenous cyclophosphamide for severe or refractory NPSLE, as the combination shows 95% response rate versus 54% with methylprednisolone alone in severe NPSLE 1

• Clinical improvement should occur within days to 3 weeks, with neurological response paralleling systemic disease activity improvement 3

Thrombosis Prevention

• Initiate low-dose aspirin (81 mg daily) for primary thrombosis prevention if antiphospholipid antibodies are positive 1

• Consider anticoagulation if imaging demonstrates thrombotic/ischemic mechanism 1

If NPSLE is Excluded: Consider Cyclic Vomiting Syndrome

The clinical pattern—episodic vomiting triggered by waking, heavy meals, and physical activity—fits cyclic vomiting syndrome (CVS), which has 2% prevalence and is frequently underdiagnosed 2:

CVS Diagnostic Features Supporting This Diagnosis

• Stereotypical episodes of acute-onset vomiting with symptom-free intervals between episodes 2

• Morning predominance of episodes is classic for CVS 2

• Triggering factors including physical activity and heavy meals are characteristic 2

• Episodes may be associated with constitutional symptoms (fatigue, anxiety, abdominal pain) without excluding CVS diagnosis 2

CVS Management Approach

While the guidelines focus on adult CVS, the principles apply to pediatric cases:

• Patient and family education on recognizing prodromal symptoms (approximately 65% experience prodromal phase lasting median 1 hour) to enable early abortive therapy 2

• Abortive therapy during prodromal phase has highest probability of successfully terminating episodes 2

• Prophylactic therapy consideration if episodes are frequent or severe (≥4 episodes/year lasting >2 days would classify as moderate-severe CVS) 2

Ongoing Monitoring Regardless of Diagnosis

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

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

• Maintain hydroxychloroquine unless contraindicated, as it reduces mortality and disease activity (dose not exceeding 5 mg/kg actual body weight) 1

• Annual ophthalmologic screening if on hydroxychloroquine 1