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Reversible cerebral vasoconstriction syndrome in paediatric patients with systemic lupus erythematosus: implications

Chloé Durrleman1, Olivier Naggara2,3,4, David Grevent3

  • 1Paediatric Neurology, Assistance Publique-Hôpitaux de Paris, Hôpital Universitaire Necker-Enfants Malades, Paris, France.

Developmental Medicine and Child Neurology
|September 25, 2018
PubMed

Insights

Reversible cerebral vasoconstriction syndrome (RCVS) was identified in three pediatric patients with systemic lupus erythematosus (SLE). Early recognition and multidisciplinary analysis are crucial for managing this rare condition in children.

Area of Science:

  • Neurology
  • Rheumatology
  • Pediatrics

Background:

  • Systemic lupus erythematosus (SLE) is known to cause microvascular brain injury.
  • Cerebral large artery involvement in pediatric SLE remains under investigation.

Observation:

  • Three female patients (ages 9-14) with SLE experienced intense headaches and neurological symptoms after immunosuppressive treatment intensification for lupus nephritis.
  • Magnetic resonance angiography revealed multiple stenoses in the circle of Willis, with one patient developing cerebral infarction.

Findings:

  • Clinical and radiological features suggested reversible cerebral vasoconstriction syndrome (RCVS) as the most likely diagnosis in all three patients.
  • Angiography normalized within weeks, indicating the reversible nature of the vasoconstriction.
  • RCVS is a rare condition in children, and this study highlights its occurrence in pediatric SLE.

Implications:

  • Multidisciplinary assessment of clinical and angiographic findings is recommended for diagnosing RCVS in pediatric SLE patients.
  • Timely recognition of RCVS in this population can guide appropriate treatment adjustments.
  • This study underscores the importance of thorough imaging analysis for diagnosing RCVS in pediatric patients.

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