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This study investigated clinical and neuropathological features, immunotherapies, and outcomes of ICU-treated CAN patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats: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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among 21 included patients (chronic inflammatory demyelinating neuropathies (CIDP):\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200915, other CAN:\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20096), 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\u2009+\u2009T-cell infiltration (9\/11), and complement deposition (C5b-9) was detected in all samples (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20098\/8). All patients received first-line immunotherapy at ICU, 62% required \u201cescalation\u201d (rituximab:\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200913, cyclophosphamide:\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093). Five (24%) remained refractory, receiving daratumumab (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093), efgartigimod (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091), or autologous stem cell transplantation (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091). Six patients (29%) died, whereas survivors showed marked improvement with median change of\u2009\u2212\u20092 mRS points (95% CI\u2009\u2212\u20095 to\u2009\u2212\u20092) and\u2009\u2212\u20096 INCAT points (95% CI\u2009\u2212\u20098 to\u2212\u20095) 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\u201327.3, INCAT 6.4 95% CI 1.7\u201323.2).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats: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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00415-026-13694-4","type":"journal-article","created":{"date-parts":[[2026,2,25]],"date-time":"2026-02-25T11:17:43Z","timestamp":1772018263000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical course and prognosis of chronic autoimmune neuropathies requiring intensive care: a retrospective cohort study"],"prefix":"10.1007","volume":"273","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3618-429X","authenticated-orcid":false,"given":"Hannah","family":"Pre\u00dfler","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1283-3478","authenticated-orcid":false,"given":"Lisa","family":"Schwarz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-0101-8081","authenticated-orcid":false,"given":"Simon","family":"Streit","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4891-8921","authenticated-orcid":false,"given":"Annette","family":"Aigner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alicia","family":"Schleicher","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5306-7880","authenticated-orcid":false,"given":"Frauke","family":"Stascheit","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0112-8685","authenticated-orcid":false,"given":"Friederike A.","family":"Arlt","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2069-4092","authenticated-orcid":false,"given":"Viktoria","family":"Zinnow","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tatjana","family":"Khorassani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8283-7976","authenticated-orcid":false,"given":"Harald","family":"Pr\u00fcss","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"B\u00f6hmerle","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andreas","family":"Meisel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Werner","family":"Stenzel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franziska","family":"Scheibe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2026,2,25]]},"reference":[{"issue":"8","key":"13694_CR1","doi-asserted-by":"publisher","first-page":"435","DOI":"10.1038\/nrneurol.2014.117","volume":"10","author":"N Latov","year":"2014","unstructured":"Latov N (2014) Diagnosis and treatment of chronic acquired demyelinating polyneuropathies. 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Arlt, Viktoria Zinnow, Tatjana Khorassani, Harald Pr\u00fc\u00df, Wolfgang B\u00f6hmerle, Werner Stenzel, and Franziska Scheibe have no competing interests to declare that are relevant to the content of this article. Frauke Stascheit received travel\/accommodation\/meeting expenses from Alexion Pharmaceuticals and argenx and received speaking honoria and honoria for attendance at advisory boards from Alexion Pharmaceuticals, argenx, and UCB pharma. She receives financial research support (paid to her institution) from Alexion Pharmaceuticals and Cytel. She serves as a member of the medical advisory of the German Myasthenia Gravis Society e.V. A.M. received speaker\u2019s speaker or consultancy honoraria or financial research support (paid to his institution) from Alexion AstraZeneca Rare Disease, Amgen, argenx, Axunio, Desitin, Genpharm, Grifols, Hormosan, Immunovant, Johneson & Johnson, Merck, Novartis, Octapharma, Regeneron, Sanofi, and UCB. He serves as member (chairmen from 2019\u20132024) of the medical advisory board of the German Myasthenia Gravis Society.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"The study was approved by the institutional ethics committee of Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin, Germany (EA2\/096\/21, EA4\/166\/23) and was performed in accordance with the Declaration of Helsinki. Generative artificial intelligence (AI) was used to improve language clarity (Chat GPT-4; Open AI). Any use of generative AI in this manuscript adheres to ethical guidelines for use and acknowledgement of generative AI in academic research [\n                      \n                      ]. This article follows the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) reporting guidelines.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}],"article-number":"165"}}