Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series
ObjectivesTo describe the occurrence of secondary sclerosing cholangitis in critically ill patients (SC-CIP) with febrile infection-related epilepsy syndrome (FIRES).MethodsMonocentric retrospective analysis of all adult patients with FIRES admitted from January 2020 to December 2024.ResultsFour pat...
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Frontiers Media S.A.
2025-04-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2025.1557377/full |
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| author | Lorenzo Muccioli Lorenzo Muccioli Lidia Di Vito Elena Pasini Lorenzo Ferri Lorenzo Ferri Giovanni Vitale Alessandro Granito Barbara Mostacci Manuel Moneti Laura Licchetta Rocco Liguori Rocco Liguori Paolo Tinuper Carlo Alberto Castioni Francesca Bisulli Francesca Bisulli |
| author_facet | Lorenzo Muccioli Lorenzo Muccioli Lidia Di Vito Elena Pasini Lorenzo Ferri Lorenzo Ferri Giovanni Vitale Alessandro Granito Barbara Mostacci Manuel Moneti Laura Licchetta Rocco Liguori Rocco Liguori Paolo Tinuper Carlo Alberto Castioni Francesca Bisulli Francesca Bisulli |
| author_sort | Lorenzo Muccioli |
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| description | ObjectivesTo describe the occurrence of secondary sclerosing cholangitis in critically ill patients (SC-CIP) with febrile infection-related epilepsy syndrome (FIRES).MethodsMonocentric retrospective analysis of all adult patients with FIRES admitted from January 2020 to December 2024.ResultsFour 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.DiscussionThe 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. |
| format | Article |
| id | doaj-art-b239063674674659b684720609aee3d1 |
| institution | OA Journals |
| issn | 1664-2295 |
| language | English |
| publishDate | 2025-04-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Neurology |
| spelling | doaj-art-b239063674674659b684720609aee3d12025-08-20T02:18:35ZengFrontiers Media S.A.Frontiers in Neurology1664-22952025-04-011610.3389/fneur.2025.15573771557377Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case seriesLorenzo Muccioli0Lorenzo Muccioli1Lidia Di Vito2Elena Pasini3Lorenzo Ferri4Lorenzo Ferri5Giovanni Vitale6Alessandro Granito7Barbara Mostacci8Manuel Moneti9Laura Licchetta10Rocco Liguori11Rocco Liguori12Paolo Tinuper13Carlo Alberto Castioni14Francesca Bisulli15Francesca Bisulli16Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyDepartment of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyInternal Medicine Unit for the Treatment of Severe Organ Failure, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, ItalyDivision of Internal Medicine, Hepatobiliary and Immunoallergic Diseases, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyDepartment of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyDepartment of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyDepartment of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, ItalyIRCCS Istituto delle Scienze Neurologiche di Bologna, Full Member of the ERN EpiCARE, Bologna, ItalyObjectivesTo describe the occurrence of secondary sclerosing cholangitis in critically ill patients (SC-CIP) with febrile infection-related epilepsy syndrome (FIRES).MethodsMonocentric retrospective analysis of all adult patients with FIRES admitted from January 2020 to December 2024.ResultsFour 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.DiscussionThe 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.https://www.frontiersin.org/articles/10.3389/fneur.2025.1557377/fullFIRESstatus epilepticusNORSEintensive careketaminedrug-induced liver injury (DILI) |
| spellingShingle | Lorenzo Muccioli Lorenzo Muccioli Lidia Di Vito Elena Pasini Lorenzo Ferri Lorenzo Ferri Giovanni Vitale Alessandro Granito Barbara Mostacci Manuel Moneti Laura Licchetta Rocco Liguori Rocco Liguori Paolo Tinuper Carlo Alberto Castioni Francesca Bisulli Francesca Bisulli Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series Frontiers in Neurology FIRES status epilepticus NORSE intensive care ketamine drug-induced liver injury (DILI) |
| title | Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series |
| title_full | Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series |
| title_fullStr | Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series |
| title_full_unstemmed | Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series |
| title_short | Secondary sclerosing cholangitis in critically ill patients with febrile infection-related epilepsy syndrome (FIRES): a case series |
| title_sort | secondary sclerosing cholangitis in critically ill patients with febrile infection related epilepsy syndrome fires a case series |
| topic | FIRES status epilepticus NORSE intensive care ketamine drug-induced liver injury (DILI) |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2025.1557377/full |
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