94% of researchers rate our articles as excellent or good
Learn more about the work of our research integrity team to safeguard the quality of each article we publish.
Find out more
BRIEF RESEARCH REPORT article
Front. Neurol.
Sec. Neurocritical and Neurohospitalist Care
Volume 16 - 2025 | doi: 10.3389/fneur.2025.1557377
Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series
Provisionally accepted
Lorenzo Muccioli1*
Lidia Di Vito2
Elena Pasini2
Lorenzo Ferri2
Giovanni Vitale3
Alessandro Granito3
Barbara Mostacci2
Manuel Moneti2Laura Licchetta2
Rocco Liguori2
Paolo TINUPER1Carlo Alberto Castioni2
Francesca Bisulli1- 1University of Bologna, Bologna, Italy
- 2IRCCS Institute of Neurological Sciences of Bologna (ISNB), Bologna, Emilia-Romagna, Italy
- 3IRCCS University Hospital of Bologna Sant Orsola Polyclinic, Bologna, Emilia-Romagna, Italy
Select one of your emails
You have multiple emails registered with Frontiers:
Notify me on publication
Please enter your email address:
If you already have an account, please login
You don't have a Frontiers account ? You can register here
Objectives: to describe the occurrence of secondary sclerosing cholangitis in critically ill patients (SC-CIP) with febrile infection-related epilepsy syndrome (FIRES).Methods: monocentric retrospective analysis of all adult patients with FIRES admitted from January 2020 to December 2024.Results: four patients (3 males) with a mean age of 24 years (range: 18-40 years) and no significant medical history presented with cryptogenic FIRES. They required treatment with antiseizure medications (mean: 9; range: 8-10), anesthetics (propofol, midazolam and ketamine in all cases), and immunotherapies. The average duration of status epilepticus (SE) was 57 days (range: 34-90 days), while the mean duration of intensive care unit (ICU) stay was 82 days (range: 58-117 days). All patients developed cholestatic liver disease during their ICU stay, reversible in one case. In the three cases with persistent injury (75%), SC-CIP was diagnosed with MR-colangiography after a mean of 106 days from SE onset.Discussion: the high incidence of SC-CIP in our cohort of patients with FIRES suggests a link between these two rare conditions, likely related to prolonged intensive care, hyperinflammation and polytherapy, including ketamine use. Vigilant monitoring of liver disease progression in critically ill patients with FIRES and similar predisposing factors may allow early recognition of SC-CIP and improved patient outcomes.
Keywords: FIRES, status epilepticus, Norse, Intensive Care, Ketamine, Drug-induced liver injury (DILI)
Received: 08 Jan 2025; Accepted: 09 Apr 2025.
Copyright: © 2025 Muccioli, Di Vito, Pasini, Ferri, Vitale, Granito, Mostacci, Moneti, Licchetta, Liguori, TINUPER, Castioni and Bisulli. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Lorenzo Muccioli, University of Bologna, Bologna, Italy
Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.