Journal Article

DZNE-2026-00243

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png Clinical course and prognosis of chronic autoimmune neuropathies requiring intensive care: a retrospective cohort study.

Preßler, H. (First author)DZNE* ; Schwarz, L. ; Streit, S. ; Aigner, A. ; Schleicher, A. ; Stascheit, F. ; Arlt, F. A.DZNE* ; Zinnow, V.DZNE* ; Khorassani, T. ; Prüss, H.DZNE* ; Böhmerle, W. ; Meisel, A. ; Stenzel, W. ; Scheibe, F.

2026
Steinkopff [Darmstadt]

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Please use a persistent id in citations: doi:10.1007/s00415-026-13694-4

Abstract: Data on chronic autoimmune neuropathies (CAN) requiring intensive care unit (ICU) treatment are limited. This study investigated clinical and neuropathological features, immunotherapies, and outcomes of ICU-treated CAN patients.Retrospectively, we analyzed patients with CAN admitted to the ICU between 2007 and 2024. Outcomes, assessed by Inflammatory Neuropathy Cause and Treatment (INCAT) score and modified Rankin scale (mRS), were compared to age, sex, and diagnosis-matched non-ICU outpatients using ordinal and binary logistic regression.Among 21 included patients (chronic inflammatory demyelinating neuropathies (CIDP): n = 15, other CAN: n = 6), 95% required mechanical ventilation and exhibited severe tetraparesis at disease nadir. Biopsies from CIDP patients revealed moderate-to-severe chronic axonal loss with variable CD8 + T-cell infiltration (9/11), and complement deposition (C5b-9) was detected in all samples (n = 8/8). All patients received first-line immunotherapy at ICU, 62% required 'escalation' (rituximab: n = 13, cyclophosphamide: n = 3). Five (24%) remained refractory, receiving daratumumab (n = 3), efgartigimod (n = 1), or autologous stem cell transplantation (n = 1). Six patients (29%) died, whereas survivors showed marked improvement with median change of - 2 mRS points (95% CI - 5 to - 2) and - 6 INCAT points (95% CI - 8 to- 5) at last follow-up. However, ICU-treated patients had significantly higher odds of worse long-term outcomes than matched non-ICU patients (adjusted cumulative OR: mRS 7.1 95% CI 1.9-27.3, INCAT 6.4 95% CI 1.7-23.2).Severe CAN requiring ICU treatment is associated with high mortality, but meaningful recovery is possible in survivors. Neuropathology confirmed combined cellular and humoral mechanisms, supporting personalized, mechanism-guided therapeutic approaches.

Keyword(s): Humans (MeSH) ; Male (MeSH) ; Female (MeSH) ; Retrospective Studies (MeSH) ; Middle Aged (MeSH) ; Aged (MeSH) ; Prognosis (MeSH) ; Critical Care (MeSH) ; Adult (MeSH) ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating: therapy (MeSH) ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating: diagnosis (MeSH) ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating: pathology (MeSH) ; Immunotherapy (MeSH) ; Intensive Care Units (MeSH) ; Autoimmune Diseases of the Nervous System: therapy (MeSH) ; Autoimmune Diseases of the Nervous System: diagnosis (MeSH) ; Autologous stem cell transplantation ; Chronic autoimmune neuropathies ; Daratumumab ; Intensive care unit

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Contributing Institute(s):

1. Autoimmune Encephalopathies (AG Prüß)

Research Program(s):

1. 353 - Clinical and Health Care Research (POF4-353) (POF4-353)

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Database coverage:
; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Life Sciences ; DEAL Springer ; DEAL Springer ; Ebsco Academic Search ; Essential Science Indicators ; IF >= 5 ; JCR ; Nationallizenz ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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