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Background and objective <p>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.</p> Methods <p>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.</p> Results <p>Among 21 included patients (chronic inflammatory demyelinating neuropathies (CIDP): <i>n</i> = 15, other CAN: <i>n</i> = 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 (<i>n</i> = 8/8). All patients received first-line immunotherapy at ICU, 62% required “escalation” (rituximab: <i>n</i> = 13, cyclophosphamide: <i>n</i> = 3). Five (24%) remained refractory, receiving daratumumab (<i>n</i> = 3), efgartigimod (<i>n</i> = 1), or autologous stem cell transplantation (<i>n</i> = 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).</p> Conclusion <p>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.</p>

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Clinical course and prognosis of chronic autoimmune neuropathies requiring intensive care: a retrospective cohort study

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  • 发表日期：

    2026年2月25日

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  • Hannah Preßler,
  • Lisa Schwarz,
  • Simon Streit,
  • Annette Aigner,
  • Alicia Schleicher,
  • Frauke Stascheit,
  • Friederike A. Arlt,
  • Viktoria Zinnow,
  • Tatjana Khorassani,
  • Harald Prüss,
  • Wolfgang Böhmerle,
  • Andreas Meisel,
  • Werner Stenzel,
  • Franziska Scheibe

摘要

Background and objective

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.

Methods

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.

Results

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).

Conclusion

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.