Open AccessAnakinra usage in febrile infection related epilepsy syndrome: an international cohort
Author(s) -
Lai YiChen,
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
Publication year - 2020
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.51229
Subject(s) - medicine , anakinra , cohort , epilepsy , intensive care medicine , cohort study , pediatrics , psychiatry , disease
Abstract 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.