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@ARTICLE{Preler:285465,
      author       = {Preßler, Hannah and Schwarz, Lisa and Streit, Simon and
                      Aigner, Annette and Schleicher, Alicia and Stascheit, Frauke
                      and Arlt, Friederike A and Zinnow, Viktoria and Khorassani,
                      Tatjana and Prüss, Harald and Böhmerle, Wolfgang and
                      Meisel, Andreas and Stenzel, Werner and Scheibe, Franziska},
      title        = {{C}linical course and prognosis of chronic autoimmune
                      neuropathies requiring intensive care: a retrospective
                      cohort study.},
      journal      = {Journal of neurology},
      volume       = {273},
      number       = {2},
      issn         = {0367-004X},
      address      = {[Darmstadt]},
      publisher    = {Steinkopff},
      reportid     = {DZNE-2026-00243},
      pages        = {165},
      year         = {2026},
      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.},
      keywords     = {Humans / Male / Female / Retrospective Studies / Middle
                      Aged / Aged / Prognosis / Critical Care / Adult /
                      Polyradiculoneuropathy, Chronic Inflammatory Demyelinating:
                      therapy / Polyradiculoneuropathy, Chronic Inflammatory
                      Demyelinating: diagnosis / Polyradiculoneuropathy, Chronic
                      Inflammatory Demyelinating: pathology / Immunotherapy /
                      Intensive Care Units / Autoimmune Diseases of the Nervous
                      System: therapy / Autoimmune Diseases of the Nervous System:
                      diagnosis / Autologous stem cell transplantation (Other) /
                      Chronic autoimmune neuropathies (Other) / Daratumumab
                      (Other) / Intensive care unit (Other)},
      cin          = {AG Prüß},
      ddc          = {610},
      cid          = {I:(DE-2719)1810003},
      pnm          = {353 - Clinical and Health Care Research (POF4-353)},
      pid          = {G:(DE-HGF)POF4-353},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:41739210},
      pmc          = {pmc:PMC12935830},
      doi          = {10.1007/s00415-026-13694-4},
      url          = {https://pub.dzne.de/record/285465},
}