Childhood central nervous system lupus; longitudinal assessment using single photon emission computed tomography

Area of Science:

• Pediatric Rheumatology
• Pediatric Neurology
• Diagnostic Imaging

Background:

• Neuropsychiatric manifestations affect 30-60% of children with systemic lupus erythematosus (SLE).
• Diagnosing central nervous system (CNS) lupus in children is challenging due to limited diagnostic tools.
• Single photon emission computed tomography (SPECT) has shown promise in assessing cerebral blood flow in active CNS lupus.

Purpose of the Study:

• To evaluate the long-term usefulness of SPECT scans in monitoring children with CNS lupus.
• To assess the correlation between SPECT findings and clinical course in pediatric CNS lupus patients over time.

Main Methods:

• An open pilot study included eleven children diagnosed with CNS lupus.
• Patients underwent initial lumbar puncture, SPECT, serologic testing, and other imaging (EEG, CT, MRI).
• SPECT scans were repeated at 1-4 months and annually for up to 3.5 years to track changes.

Main Results:

• All patients (100%) showed abnormal SPECT scans during the initial CNS event, unlike other diagnostic tests.
• Follow-up SPECT scans revealed persistent abnormalities, with perfusion defects improving in 4 patients and worsening in 6.
• Despite clinical resolution of CNS manifestations, SPECT abnormalities did not normalize over the long term.

Conclusions:

• SPECT is a sensitive tool for initial diagnosis of CNS lupus in children, detecting cerebral damage.
• Long-term SPECT abnormalities in pediatric CNS lupus do not correlate with the patient's clinical recovery.
• The utility of SPECT as a clinical tool for monitoring children with CNS lupus is limited in long-term follow-up.