BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an inflammatory disease affecting the peripheral nerves and the most frequent autoimmune polyneuropathy. Given the lack of established biomarkers or risk factors for the development of CIDP and patients' treatment response, this research effort seeks to identify potential clinical factors that may influence disease progression and overall treatment efficacy. METHODS: In this multicenter, retrospective analysis, we have screened 197 CIDP patients who presented to the University Hospitals in Düsseldorf, Berlin, Cologne, Essen, Magdeburg and Munich between 2018 and 2022. We utilized the respective hospital information system and examined baseline data with clinical examination, medical letters, laboratory results, antibody status, nerve conduction studies, imaging and biopsy findings. Aside from clinical baseline data, we analyzed treatment outcomes using the Standard of Care (SOC) definition, as well as a comparison of an early (within the first 12 months after manifestation) versus late (more than 12 months after manifestation) onset of therapy. RESULTS: In terms of treatment, most patients received intravenous immunoglobulin (56%) or prednisolone (39%) as their first therapy. Patients who started their initial treatment later experienced a worsening disease course, as reflected by a significant deterioration in their Inflammatory Neuropathy Cause and Treatment (INCAT) leg disability score. SOC-refractory patients had worse clinical outcomes than SOC-responders. Associated factors for SOC-refractory status included the presence of fatigue as a symptom and alcohol dependence. CONCLUSION: Timely diagnosis, prompt initiation of treatment and careful monitoring of treatment response are essential for the prevention of long-term disability in CIDP and suggest a "hit hard and early" treatment paradigm.

Brain and Mind Center University of Sydney 94 Mallett St Sydney Australia

Center for Behavioral Brain Sciences Otto von Guericke University 39106 Magdeburg Germany

Center for Translational Neuro and Behavioral Sciences University of Duisburg Essen Essen Germany

Department of Neurology and Experimental Neurology Charité Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin Berlin Germany

Department of Neurology Essen University Hospital University Duisburg Essen Essen Germany

Department of Neurology Faculty of Medicine University of Cologne and University Hospital of Cologne Cologne Germany

Department of Neurology Klinikum Rechts der Isar Technical University Munich School of Medicine and Health Ismaninger Str 22 81675 Munich Germany

Department of Neurology Medical Faculty and University Hospital Düsseldorf Heinrich Heine University Düsseldorf Moorenstr 5 40225 Duesseldorf Germany

Department of Neurology Otto von Guericke University 39120 Magdeburg Germany

Department of Neurology Palacky University Olomouc Nová Ulice 779 00 Olomouc Czech Republic

Department of Orthopaedics and Trauma Surgery Medical Faculty Heinrich Heine University Duesseldorf Moorenstr 5 40225 Duesseldorf Germany

German Center for Neurodegenerative Diseases 39120 Magdeburg Germany

Institute for Experimental Neuroimmunology Technical University of Munich School of Medicine and Health Munich Germany

Munich Cluster for Systems Neurology 81377 Munich Germany

Neuroscience Clinical Research Center Charité Universitätsmedizin Berlin corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin Berlin Germany

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Broers MC et al (2019) Incidence and prevalence of chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis. Neuroepidemiology 52(3–4):161–172 10.1159/000494291 PubMed DOI PMC

Van den Bergh PY, Rajabally YA (2013) Chronic inflammatory demyelinating polyradiculoneuropathy. Presse Med 42(6 Pt 2):e203–e215 10.1016/j.lpm.2013.01.056 PubMed DOI

Mathey EK et al (2015) Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry 86(9):973–985 10.1136/jnnp-2014-309697 PubMed DOI PMC

Köller H et al (2005) Chronic inflammatory demyelinating polyneuropathy. N Engl J Med 352(13):1343–1356 10.1056/NEJMra041347 PubMed DOI

Querol L et al (2017) Antibodies against peripheral nerve antigens in chronic inflammatory demyelinating polyradiculoneuropathy. Sci Rep 7(1):14411 10.1038/s41598-017-14853-4 PubMed DOI PMC

Devaux JJ, Odaka M, Yuki N (2012) Nodal proteins are target antigens in Guillain-Barre syndrome. J Peripher Nerv Syst 17(1):62–71 10.1111/j.1529-8027.2012.00372.x PubMed DOI

Cifuentes-Diaz C et al (2011) Nodes of ranvier and paranodes in chronic acquired neuropathies. PLoS ONE 6(1):e14533 10.1371/journal.pone.0014533 PubMed DOI PMC

Delmont E et al (2017) Autoantibodies to nodal isoforms of neurofascin in chronic inflammatory demyelinating polyneuropathy. Brain 140(7):1851–1858 10.1093/brain/awx124 PubMed DOI

Liberatore G et al (2022) Frequency and clinical correlates of anti-nerve antibodies in a large population of CIDP patients included in the Italian database. Neurol Sci 43(6):3939–3947 10.1007/s10072-021-05811-0 PubMed DOI

Tang L et al (2021) Distinguish CIDP with autoantibody from that without autoantibody: pathogenesis, histopathology, and clinical features. J Neurol 268(8):2757–2768 10.1007/s00415-020-09823-2 PubMed DOI

Van den Bergh PYK et al (2021) European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint Task Force-Second revision. Eur J Neurol 28(11):3556–3583 10.1111/ene.14959 PubMed DOI

Querol L et al (2021) Systematic literature review of burden of illness in chronic inflammatory demyelinating polyneuropathy (CIDP). J Neurol 268(10):3706–3716 10.1007/s00415-020-09998-8 PubMed DOI PMC

Kuwabara S, Misawa S (2019) Chronic inflammatory demyelinating polyneuropathy. Adv Exp Med Biol 1190:333–343 10.1007/978-981-32-9636-7_21 PubMed DOI

Doneddu PE et al (2019) Atypical CIDP: diagnostic criteria, progression and treatment response. Data from the Italian CIDP Database. J Neurol Neurosurg Psychiatry 90(2):125–132 10.1136/jnnp-2018-318714 PubMed DOI

Menon D, Katzberg HD, Bril V (2021) Treatment approaches for Atypical CIDP. Front Neurol 12:653734 10.3389/fneur.2021.653734 PubMed DOI PMC

Bunschoten C et al (2019) Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy. Lancet Neurol 18(8):784–794 10.1016/S1474-4422(19)30144-9 PubMed DOI

Wieske L et al (2023) Proximity extension assay-based discovery of biomarkers for disease activity in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 95:595–60410.1136/jnnp-2023-332398 PubMed DOI

Oeztuerk M et al (2023) Current biomarker strategies in autoimmune neuromuscular diseases. Cells 12(20):2456 10.3390/cells12202456 PubMed DOI PMC

Stascheit F et al (2021) Calprotectin in chronic inflammatory demyelinating polyneuropathy and variants-a potential novel biomarker of disease activity. Front Neurol 12:723009 10.3389/fneur.2021.723009 PubMed DOI PMC

Mausberg AK et al (2020) NK cell markers predict the efficacy of IV immunoglobulins in CIDP. Neurol Neuroimmunol Neuroinflamm. 10.1212/NXI.0000000000000884 10.1212/NXI.0000000000000884 PubMed DOI PMC

Stettner M et al (2016) Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy. Ann Clin Transl Neurol 3(2):88–100 10.1002/acn3.275 PubMed DOI PMC

Doneddu PE et al (2020) Risk factors for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): antecedent events, lifestyle and dietary habits. Data from the Italian CIDP Database. Eur J Neurol 27(1):136–143 10.1111/ene.14044 PubMed DOI

Lehmann HC, Burke D, Kuwabara S (2019) Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment. J Neurol Neurosurg Psychiatry 90(9):981–987 10.1136/jnnp-2019-320314 PubMed DOI

Stino AM et al (2021) Chronic inflammatory demyelinating polyradiculoneuropathy—diagnostic pitfalls and treatment approach. Muscle Nerve 63(2):157–169 10.1002/mus.27046 PubMed DOI

Allen JA (2021) Measuring treatment response to chronic inflammatory demyelinating polyneuropathy in clinical practice: more than just asking. Muscle Nerve 64(1):1–3 10.1002/mus.27325 PubMed DOI

Katzberg HD, Latov N, Walker FO (2017) Measuring disease activity and clinical response during maintenance therapy in CIDP: from ICE trial outcome measures to future clinical biomarkers. Neurodegener Dis Manag 7(2):147–156 10.2217/nmt-2016-0058 PubMed DOI

Hughes R et al (2001) Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol 50(2):195–201 10.1002/ana.1088 PubMed DOI

Kleyweg RP, van der Meche FG, Schmitz PI (1991) Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain-Barre syndrome. Muscle Nerve 14(11):1103–1109 10.1002/mus.880141111 PubMed DOI

Oaklander AL et al (2017) Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews. Cochrane Database Syst Rev 1(1):CD010369 PubMed PMC

Cocito D et al (2010) A nationwide retrospective analysis on the effect of immune therapies in patients with chronic inflammatory demyelinating polyradiculoneuropathy. Eur J Neurol 17(2):289–294 10.1111/j.1468-1331.2009.02802.x PubMed DOI

Robertson EE, Donofrio PD (2010) Treatment of chronic inflammatory demyelinating polyneuropathy. Curr Treat Options Neurol 12(2):84–94 10.1007/s11940-010-0058-9 PubMed DOI

McCombe PA et al (2022) Sex differences in Guillain Barre syndrome, chronic inflammatory demyelinating polyradiculoneuropathy and experimental autoimmune neuritis. Front Immunol. 10.3389/fimmu.2022.1038411 10.3389/fimmu.2022.1038411 PubMed DOI PMC

Abraham A et al (2016) Frequent laboratory abnormalities in CIDP patients. Muscle Nerve 53(6):862–865 10.1002/mus.24978 PubMed DOI

Eftimov F et al (2020) Diagnostic challenges in chronic inflammatory demyelinating polyradiculoneuropathy. Brain 143(11):3214–3224 10.1093/brain/awaa265 PubMed DOI PMC

Allen JA, Lewis RA (2022) Diagnosis of chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 66(5):545–551 10.1002/mus.27708 PubMed DOI

Gable KL et al (2022) A longitudinal evaluation of fatigue in chronic inflammatory demyelinating polyneuropathy. Brain Behav 12(8):e2712 10.1002/brb3.2712 PubMed DOI PMC