Anakinra usage in febrile infection related epilepsy syndrome: an international cohort

Febrile‐infection related epilepsy syndrome (FIRES) is a devastating neurological condition characterized by a febrile illness preceding new onset refractory status epilepticus (NORSE). Increasing evidence suggests innate immune dysfunction as a potential pathological mechanism. We report an interna...

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Bibliographic Details
Published in:Annals of clinical and translational neurology 2020-12, Vol.7 (12), p.2467-2474
Main Authors: Lai, Yi‐Chen, Muscal, Eyal, Wells, Elizabeth, Shukla, Nikita, Eschbach, Krista, Hyeong Lee, Ki, Kaliakatsos, Marios, Desai, Nevedita, Wickström, Ronny, Viri, Maurizio, Freri, Elena, Granata, Tiziana, Nangia, Srishti, Dilena, Robertino, Brunklaus, Andreas, Wainwright, Mark S., Gorman, Mark P., Stredny, Coral M., Asiri, Abdurhman, Hundallah, Khalid, Doja, Asif, Payne, Eric, Wirrell, Elaine, Koh, Sookyong, Carpenter, Jessica L., Riviello, James
Format: Article
Language:English
Subjects:
Autoimmune diseases
Brief Communication
Brief Communications
Convulsions & seizures
Cytokines
Demographics
Encephalitis
Epilepsy
Hispanic people
Hypothermia
Infections
Medicin och hälsovetenskap
Pediatrics
Proteins
Tumor necrosis factor-TNF
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Summary:Febrile‐infection related epilepsy syndrome (FIRES) is a devastating neurological condition characterized by a febrile illness preceding new onset refractory status epilepticus (NORSE). Increasing evidence suggests innate immune dysfunction as a potential pathological mechanism. We report an international retrospective cohort of 25 children treated with anakinra, a recombinant interleukin‐1 receptor antagonist, as an immunomodulator for FIRES. Anakinra was potentially safe with only one child discontinuing therapy due to infection. Earlier anakinra initiation was associated with shorter duration of mechanical ventilation, ICU and hospital length of stay. Our retrospective data lay the groundwork for prospective consensus‐driven cohort studies of anakinra in FIRES.
ISSN:2328-9503
2328-9503
DOI:10.1002/acn3.51229