Anti-Neuronal, Anti-Ribosomal P And
Anti-Phospholipid Antibodies In Pediatric
Neuropsychiatric Lupus
Eyal Muscal, Anna A. Minta, and Barry L. Myones
Baylor College of Medicine
Houston, TX
Texas
C h ild ren ’s
H osp ita l
Background
• Neuropsychiatric syndromes are reported in 20-95% of
pediatric lupus patients
• Understanding of neurologic dysfunction in pediatric
lupus is based on adult experience and research
• Postulated immune responses against phospholipid,
neuronal, and/or ribosomal associated autoantigens
• Paucity of data on immune dysregulation and
autoantibody effects in pediatric neuropsychiatric
lupus
Background
• Anti-phospholipid antibodies associated with vasculopathy and
focal CNS events
• Anti-phospholipid antibodies and lupus anti-coagulants may be
involved in neuronal cell death and diffuse damage
• Animal model administration of anti-neuronal antibodies induces
cognitive impairments, and seizures
• Anti-ribosomal P antibodies proposed to act as anti-endothelial cell
antibodies
Purpose and Hypothesis
• Describe prevalence rates of anti-phospholipid, anti-
neuronal and anti-ribosomal P antibodies in pediatric lupus
• Characterize associations between CNS specific
autoantibodies and pediatric neuropsychiatric lupus
• Postulate increased incidence of neuropsychiatric events
in patients exposed to multiple CNS specific
autoantibodies
Patients and Methods
• The records of 150 patients with pediatric lupus seen over a 10
year period were reviewed in a retrospective fashion
• Autoantibody status and evidence of neuropsychiatric events were
obtained via review of outpatient charts, electronic medical record
and laboratory values database
• Documentation of nervous system events based on revised 1999
ACR neuropsychiatric lupus nomenclature
Patients and Methods
• Anti-phospholipid antibody status was based on comprehensive
panel performed via a β-2-glycoprotein-1 dependent assay
• Lupus anti-coagulant testing: PTT-LA, dilute Russell viper venom
test and hexagonal-phase pho