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000285465 1001_ $$0P:(DE-2719)9003388$$aPreßler, Hannah$$b0$$eFirst author
000285465 245__ $$aClinical course and prognosis of chronic autoimmune neuropathies requiring intensive care: a retrospective cohort study.
000285465 260__ $$a[Darmstadt]$$bSteinkopff$$c2026
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000285465 520__ $$aData on chronic autoimmune neuropathies (CAN) requiring intensive care unit (ICU) treatment are limited. This study investigated clinical and neuropathological features, immunotherapies, and outcomes of ICU-treated CAN patients.Retrospectively, we analyzed patients with CAN admitted to the ICU between 2007 and 2024. Outcomes, assessed by Inflammatory Neuropathy Cause and Treatment (INCAT) score and modified Rankin scale (mRS), were compared to age, sex, and diagnosis-matched non-ICU outpatients using ordinal and binary logistic regression.Among 21 included patients (chronic inflammatory demyelinating neuropathies (CIDP): n = 15, other CAN: n = 6), 95% required mechanical ventilation and exhibited severe tetraparesis at disease nadir. Biopsies from CIDP patients revealed moderate-to-severe chronic axonal loss with variable CD8 + T-cell infiltration (9/11), and complement deposition (C5b-9) was detected in all samples (n = 8/8). All patients received first-line immunotherapy at ICU, 62% required 'escalation' (rituximab: n = 13, cyclophosphamide: n = 3). Five (24%) remained refractory, receiving daratumumab (n = 3), efgartigimod (n = 1), or autologous stem cell transplantation (n = 1). Six patients (29%) died, whereas survivors showed marked improvement with median change of - 2 mRS points (95% CI - 5 to - 2) and - 6 INCAT points (95% CI - 8 to- 5) at last follow-up. However, ICU-treated patients had significantly higher odds of worse long-term outcomes than matched non-ICU patients (adjusted cumulative OR: mRS 7.1 95% CI 1.9-27.3, INCAT 6.4 95% CI 1.7-23.2).Severe CAN requiring ICU treatment is associated with high mortality, but meaningful recovery is possible in survivors. Neuropathology confirmed combined cellular and humoral mechanisms, supporting personalized, mechanism-guided therapeutic approaches.
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000285465 650_7 $$2Other$$aAutologous stem cell transplantation
000285465 650_7 $$2Other$$aChronic autoimmune neuropathies
000285465 650_7 $$2Other$$aDaratumumab
000285465 650_7 $$2Other$$aIntensive care unit
000285465 650_2 $$2MeSH$$aHumans
000285465 650_2 $$2MeSH$$aMale
000285465 650_2 $$2MeSH$$aFemale
000285465 650_2 $$2MeSH$$aRetrospective Studies
000285465 650_2 $$2MeSH$$aMiddle Aged
000285465 650_2 $$2MeSH$$aAged
000285465 650_2 $$2MeSH$$aPrognosis
000285465 650_2 $$2MeSH$$aCritical Care
000285465 650_2 $$2MeSH$$aAdult
000285465 650_2 $$2MeSH$$aPolyradiculoneuropathy, Chronic Inflammatory Demyelinating: therapy
000285465 650_2 $$2MeSH$$aPolyradiculoneuropathy, Chronic Inflammatory Demyelinating: diagnosis
000285465 650_2 $$2MeSH$$aPolyradiculoneuropathy, Chronic Inflammatory Demyelinating: pathology
000285465 650_2 $$2MeSH$$aImmunotherapy
000285465 650_2 $$2MeSH$$aIntensive Care Units
000285465 650_2 $$2MeSH$$aAutoimmune Diseases of the Nervous System: therapy
000285465 650_2 $$2MeSH$$aAutoimmune Diseases of the Nervous System: diagnosis
000285465 7001_ $$0P:(DE-HGF)0$$aSchwarz, Lisa$$b1
000285465 7001_ $$00009-0008-0101-8081$$aStreit, Simon$$b2
000285465 7001_ $$00000-0003-4891-8921$$aAigner, Annette$$b3
000285465 7001_ $$aSchleicher, Alicia$$b4
000285465 7001_ $$00000-0001-5306-7880$$aStascheit, Frauke$$b5
000285465 7001_ $$0P:(DE-2719)9002282$$aArlt, Friederike A$$b6
000285465 7001_ $$0P:(DE-2719)9003096$$aZinnow, Viktoria$$b7$$udzne
000285465 7001_ $$aKhorassani, Tatjana$$b8
000285465 7001_ $$0P:(DE-2719)2810931$$aPrüss, Harald$$b9
000285465 7001_ $$aBöhmerle, Wolfgang$$b10
000285465 7001_ $$aMeisel, Andreas$$b11
000285465 7001_ $$aStenzel, Werner$$b12
000285465 7001_ $$aScheibe, Franziska$$b13
000285465 773__ $$0PERI:(DE-600)1421299-7$$a10.1007/s00415-026-13694-4$$gVol. 273, no. 2, p. 165$$n2$$p165$$tJournal of neurology$$v273$$x0367-004X$$y2026
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