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

• Type of Material: Article
• Authors: Yi-Chen Lai, Baylor College of Medicine/Texas Children's Hospital; Eyal Muscal, Baylor College of Medicine/Texas Children's Hospital; Elizabeth Wells, Children's National Health System; Nikita Shukla, Baylor College of Medicine/Texas Children's Hospital; Krista Eschbach, University of Colorado; Ki Hyeong Lee, AdventHealth; Mairos Kaliakatsos, Great Ormond Street Hospital; Nevedita Desai, Great Ormond Street Hospital; Ronny Wickstrom, Karolinska University Hospital; Maurizio Viri, University Hospital Maggiore della Carità; Elena Freri, FONDAZIONE IRCCS ISTITUTO NEUROLOGICO "CARLO BESTA"; Tiziana Granata, FONDAZIONE IRCCS ISTITUTO NEUROLOGICO "CARLO BESTA"; Srishti Nangia, New York Presbyterian Hospital‐Weill Cornell Medical; Robertino Dilena, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico; Andreas Brunklaus, Royal Hospital for Children; Mark S. Wainwright, University of Washington; Mark P. Gorman, Harvard Medical School; Coral M. Stredny, Harvard Medical School; Abdurhman Asiri, Prince Sultan Medical Military City (PSMMC); Khalid Hundallah, Prince Sultan Medical Military City (PSMMC); Asif Doja, University of Ottawa; Eric Payne, Alberta Children’s Hospital; Elaine Wirrell, Mayo Clinic; Sookyong Koh, Emory University; Jessica L. Carpenter, Children's National Health System; James Riviello, Baylor College of Medicine/Texas Children's Hospital
• Language: English
• Date: 2020-12-04
• Publisher: WILEY
• Publication Version: Final Published Version
• Copyright Statement: © 2020 The Authors.
• License: Creative Commons Attribution 4.0 International
• Final Published Version (URL): http://dx.doi.org/10.1002/acn3.51229
• Title of Journal or Parent Work: ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
• Volume: 7
• Issue: 12
• Start Page: 2467
• End Page: 2474
• Keywords: SYNDROME FIRES; Clinical Neurology; Life Sciences & Biomedicine; Neurosciences & Neurology; Neurosciences; Science & Technology
• Research Categories: Engineering, Biomedical; Biology, Neuroscience

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.