Clinical characteristics, treatments, outcome, and prognostic factors of severe autoimmune encephalitis in the intensive care unit: Standard treatment and the value of additional plasma cell-depleting escalation therapies for treatment-refractory patients.
autoimmune encephalitis
bortezomib
daratumumab
intensive care unit
plasma cell depletion
Journal
European journal of neurology
ISSN: 1468-1331
Titre abrégé: Eur J Neurol
Pays: England
ID NLM: 9506311
Informations de publication
Date de publication:
02 2023
02 2023
Historique:
revised:
20
09
2022
received:
25
07
2022
accepted:
23
09
2022
pubmed:
1
10
2022
medline:
7
1
2023
entrez:
30
9
2022
Statut:
ppublish
Résumé
To investigate severe autoimmune encephalitis (AE) in the intensive care unit (ICU) with regard to standard treatment in responsive patients and additional escalation therapies for treatment-refractory cases. This retrospective, single-center study analyzed medical records of ICU-dependent AE patients for clinical characteristics, treatments, prognostic factors, and neurological outcome as quantified by modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE). From 40 enrolled patients (median age = 52 years; range = 16-89 years) with AE mediated by neuronal surface antibodies (nsAb; 90%) and AE with onconeuronal antibodies (10%), 98% received first-line therapy. Of those, 62% obtained additional second-line therapy, and 33% received escalation therapy with bortezomib and/or daratumumab. Good neurological outcome, defined as mRS = 0-2, was observed in 47% of AE with nsAb (CASE = 5), 77% of anti-N-methyl D-aspartate receptor encephalitis patients (CASE = 1), whereas AE patients with onconeuronal antibodies had the poorest outcome (mRS = 6, 100%). Treatment-refractory AE patients with nsAb requiring escalation therapy achieved similarly good recovery (mRS = 0-2, 39%, CASE = 3) as patients improving without (mRS = 0-2, 54%, CASE = 4), although they presented a higher disease severity at disease maximum (mRS = 5 100% versus 68%, CASE = 24 versus 17; p = 0.0036), had longer ICU stays (97 versus 23 days; p = 0.0002), and a higher survival propability during follow-up (p = 0.0203). Prognostic factors for good recovery were younger age (p = 0.025) and lack of preexisting comorbidities (p = 0.011). Our findings suggest that treatment-refractory AE patients with nsAb in the ICU can reach similarly good outcomes after plasma cell-depleting escalation therapy as patients already responding to standard first- and/or second-line therapies.
Sections du résumé
BACKGROUND AND PURPOSE
To investigate severe autoimmune encephalitis (AE) in the intensive care unit (ICU) with regard to standard treatment in responsive patients and additional escalation therapies for treatment-refractory cases.
METHODS
This retrospective, single-center study analyzed medical records of ICU-dependent AE patients for clinical characteristics, treatments, prognostic factors, and neurological outcome as quantified by modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE).
RESULTS
From 40 enrolled patients (median age = 52 years; range = 16-89 years) with AE mediated by neuronal surface antibodies (nsAb; 90%) and AE with onconeuronal antibodies (10%), 98% received first-line therapy. Of those, 62% obtained additional second-line therapy, and 33% received escalation therapy with bortezomib and/or daratumumab. Good neurological outcome, defined as mRS = 0-2, was observed in 47% of AE with nsAb (CASE = 5), 77% of anti-N-methyl D-aspartate receptor encephalitis patients (CASE = 1), whereas AE patients with onconeuronal antibodies had the poorest outcome (mRS = 6, 100%). Treatment-refractory AE patients with nsAb requiring escalation therapy achieved similarly good recovery (mRS = 0-2, 39%, CASE = 3) as patients improving without (mRS = 0-2, 54%, CASE = 4), although they presented a higher disease severity at disease maximum (mRS = 5 100% versus 68%, CASE = 24 versus 17; p = 0.0036), had longer ICU stays (97 versus 23 days; p = 0.0002), and a higher survival propability during follow-up (p = 0.0203). Prognostic factors for good recovery were younger age (p = 0.025) and lack of preexisting comorbidities (p = 0.011).
CONCLUSIONS
Our findings suggest that treatment-refractory AE patients with nsAb in the ICU can reach similarly good outcomes after plasma cell-depleting escalation therapy as patients already responding to standard first- and/or second-line therapies.
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
474-489Commentaires et corrections
Type : CommentIn
Informations de copyright
© 2022 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.
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