Preventing long-term disability in CIDP: the role of timely diagnosis and treatment monitoring in a multicenter CIDP cohort.

Autor: Quint P; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Schroeter CB; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Kohle F; Department of Neurology, Faculty of Medicine, University of Cologne and University Hospital of Cologne, Cologne, Germany., Öztürk M; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Meisel A; Department of Neurology and Experimental Neurology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin, Berlin, Germany.; Neuroscience Clinical Research Center, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin, Berlin, Germany., Tamburrino G; Department of Neurology, Faculty of Medicine, University of Cologne and University Hospital of Cologne, Cologne, Germany., Mausberg AK; Department of Neurology, Essen University Hospital, University Duisburg-Essen, Essen, Germany.; Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University of Duisburg-Essen, Essen, Germany., Szepanowski F; Department of Neurology, Essen University Hospital, University Duisburg-Essen, Essen, Germany.; Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University of Duisburg-Essen, Essen, Germany., Afzali AM; Department of Neurology, Klinikum Rechts der Isar, Technical University Munich School of Medicine and Health, Ismaninger Str. 22, 81675, Munich, Germany.; Institute for Experimental Neuroimmunology, Technical University of Munich School of Medicine and Health, Munich, Germany., Fischer K; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Nelke C; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Räuber S; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Voth J; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Masanneck L; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Willison A; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Vogelsang A; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Hemmer B; Department of Neurology, Klinikum Rechts der Isar, Technical University Munich School of Medicine and Health, Ismaninger Str. 22, 81675, Munich, Germany.; Munich Cluster for Systems Neurology (SyNergy), 81377, Munich, Germany., Berthele A; Department of Neurology, Klinikum Rechts der Isar, Technical University Munich School of Medicine and Health, Ismaninger Str. 22, 81675, Munich, Germany., Schroeter M; Department of Neurology, Faculty of Medicine, University of Cologne and University Hospital of Cologne, Cologne, Germany., Hartung HP; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany.; Brain and Mind Center, University of Sydney, 94 Mallett St, Sydney, Australia.; Department of Neurology, Palacky University Olomouc, Nová Ulice, 779 00, Olomouc, Czech Republic., Pawlitzki M; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Schreiber S; 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.; German Center for Neurodegenerative Diseases (DZNE), 39120, Magdeburg, Germany.; Center for Behavioral Brain Sciences (CBBS), Otto-von-Guericke University, 39106, Magdeburg, Germany., Stettner M; Department of Neurology, Essen University Hospital, University Duisburg-Essen, Essen, Germany.; Center for Translational Neuro- and Behavioral Sciences (C-TNBS), University of Duisburg-Essen, Essen, Germany., Maus U; Department of Orthopaedics and Trauma Surgery, Medical Faculty, Heinrich Heine University Duesseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Meuth SG; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany., Stascheit F; Department of Neurology and Experimental Neurology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin, Berlin, Germany.; Neuroscience Clinical Research Center, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt Universität zu Berlin, Berlin, Germany., Ruck T; Department of Neurology, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University Düsseldorf, Moorenstr. 5, 40225, Duesseldorf, Germany. Tobias.Ruck@med.uni-duesseldorf.de.
Jazyk: angličtina
Zdroj: Journal of neurology [J Neurol] 2024 Sep; Vol. 271 (9), pp. 5930-5943. Date of Electronic Publication: 2024 Jul 11.
DOI: 10.1007/s00415-024-12548-1
Abstrakt: 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.
(© 2024. The Author(s).)
Databáze: MEDLINE
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