Reversible cerebral vasoconstriction syndrome in paediatric patients with systemic lupus erythematosus: implications for management. (23rd September 2018)

• Record Type: Journal Article
• Authors: Durrleman, Chloé; Naggara, Olivier; Grevent, David; Belot, Alexandre; Desgranges, Marie; Boyer, Olivia; Chabrier, Stéphane; Bader‐Meunier, Brigitte; Kossorotoff, Manoelle
• Is Part Of: Developmental medicine & child neurology. Volume 61:Number 6(2019)
• Page Start: 725
• Page End: 729
• Publication Date: 2018-09-23
• Subjects: Child development -- Periodicals; Pediatric neurology -- Periodicals; 616.8
• DOI: 10.1111/dmcn.14031
• Languages: English
• ISSNs: 0012-1622

Abstract

Abstract : 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. 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. This article's abstract has been translated into Spanish and Portuguese. Follow the links from the abstract to view the translations. This article is commented on by Dlamini on page 630 of this issue. Resumen: Síndrome de vasoconstricción cerebral reversible en pacientes pediátricos con lupus eritematoso sistémico: implicancias para el manejo: La lesión microvascular cerebral está bien reconocida en el Lupus eritematoso sistémico neuropsiquiátrico (LES), pero se debate la participación de las arterias cerebrales grandes. Tres mujeres con LES, de edades entre 9 y 14 años, tuvieron una intensificación del tratamiento inmunosupresión por la nefritis lúpica. Dentro de los siguientes días o semanas, presentaron cefalea intensa, aislada o asociada con síntomas neurológicos ‐ con o sin leve hipertensión. La angiografía por resonancia magnética mostro múltiples estenosis en el círculo de Willis. Un paciente tuvo subsecuentemente un pequeño infarto cerebral subcortical. Resumo: Síndrome da vasoconstrição cerebral reversível em pacientes pediátricos com lupus eritomatoso sistêmico: implicações para o manejo: Lesão cerebral microvascular é bem reconhecida no lupus eritomatoso sistêmico (LES) neuropsiquiátrico, mas o envolvimento arterial cerebral extenso tem sido debatido. Três meninas com LES, idades de 9 a 14 anos, tiveram intensificação do tratamento imunossupressivo devido a nefrite causada pelo lupus. Dentro dos dias ou semanas seguintes, apresentaram cefalalgia intensa – isolada ou associada com sintomas neurológicos – sem nenhuma, ou com leve hipertensão. A angiografia por ressonância magnética mostrou estenoses múltiplas no círculo de Willis. Uma paciente teve subsequente infarto cerebral subcortical pequeno. Duas pacientes foram tratadas para LES neuropsiquiátrico, uma foi tratada para síndrome da vasoconstrição cerebral reversível (SVCR). A angiografia se normalizou em algumas semanas em todas as três pacientes. Retrospectivamente, os achados clínicos e radiológicos sugerem que SVCR era o diagnóstico mais provável em todas. Análise multidisciplinar dos aspectos clínicos e angiográficos é recomendada, pois a SVCR é rara em crianças, e seu reconhecimento pode ajudar a ajustar o tratamento.