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Reversible cerebral vasoconstriction syndrome in paediatric patients with systemic lupus erythematosus: implications for management
Author(s) -
Durrleman Chloé,
Naggara Olivier,
Grevent David,
Belot Alexandre,
Desgranges Marie,
Boyer Olivia,
Chabrier Stéphane,
BaderMeunier Brigitte,
Kossorotoff Manoelle
Publication year - 2019
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/dmcn.14031
Subject(s) - medicine , reversible cerebral vasoconstriction syndrome , systemic lupus erythematosus , antiphospholipid syndrome , vasoconstriction , magnetic resonance angiography , pediatrics , magnetic resonance imaging , cardiology , radiology , thrombosis , disease
Microvascular brain injury is well recognized in neuropsychiatric systemic lupus erythematosus ( SLE ), but cerebral large artery involvement is being debated. Three females with SLE , aged 9 to 14 years, had immunosuppressive treatment intensification because of lupus nephritis. Within the following days or weeks, they presented with intense cephalalgia – isolated or associated with neurological symptoms – and no or mild hypertension. Magnetic resonance angiography showed multiple stenoses within the circle of Willis. One patient had subsequent small subcortical cerebral infarction. Two patients were treated for neuropsychiatric SLE ; one patient was treated for reversible cerebral vasoconstriction syndrome ( RCVS ). Angiography normalized within a few weeks in all three patients. Retrospectively, clinical and radiological features suggest that RCVS was the most likely diagnosis in all patients. Multidisciplinary analysis of clinical and angiographic features is recommended, as RCVS is rare in children and its recognition may help to adjust treatment. What this paper adds Reversible vasoconstriction syndrome was observed in paediatric systemic lupus erythematosus. Thorough imaging analysis was necessary to address this diagnosis in paediatric patients.