The Long-Term Course of Chronic Inflammatory Demyelinating Polyneuropathy: a Retrospective Study
- Authors: Melnik E.A.1, Arestova A.S.1, Berdalina I.A.1, Gnedovskaya E.V.1, Grishinа D.A.1, Suponeva N.A.1, Piradov M.A.1
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Affiliations:
- Research Center of Neurology
- Issue: Vol 17, No 4 (2023)
- Pages: 5-16
- Section: Original articles
- URL: https://journals.rcsi.science/2075-5473/article/view/251934
- DOI: https://doi.org/10.54101/ACEN.2023.4.1
- ID: 251934
Cite item
Abstract
Introduction. Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by long-term progressive or relapsing course, neurological deficit, and disability of varied severity. The course of CIDP after specific therapy and, if necessary, long-term maintenance treatment are to be studied.
Objective: To evaluate CIDP clinical and history characteristics over the long-term follow-up (> 5 years), to compare long-term CIDP course in a number of clinical variants and onset types, and to determine clinical predictors of unfavorable CIDP course.
Materials and methods. The study included 45 patients diagnosed with CIDP based on EAN/PNS 2021 criteria lasting for 5 or more years. Retrospective collection and analysis of medical records and clinical history were performed. Internationally accepted scales were used to assess neurological deficit (NIS, MRCss), disability (INCAT), and disease activity status (CDAS). The criteria of unfavorable course were developed to evaluate factors affecting CIDP course.
Results. Among the patients with CIDP history of >5 years, each third (34%) had no neurological deficit and remained in long-term clinical remission (CDAS 1). The vast majority (90%) responded to first-line therapy in early disease, while only 53% of patients required maintenance treatment in 5 or more years of the onset. With the developed criteria (poor response to glucocorticosteroids (GCS), need for maintenance therapy, and CDAS 3–5), unfavourable CIDP course was detected in 24 (53.3%) participants. Its probability increased in later onset (47 [30; 50] years), the chronic type of onset, and delayed specific therapy. The most significant predictors included low total NIS score at onset (<60 points) and multifocal CIDP.
Conclusions. The course of typical CIDP is relatively favorable if timely diagnosed, and pathogenetic treatment initiated. Patients with acute and subacute onset demonstrate the best long-term status. The predictors of unfavourable disease course include mild neurological deficit at onset (NIS total score <60 points) and multifocal CIDP.
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##article.viewOnOriginalSite##About the authors
Evgeniya A. Melnik
Research Center of Neurology
Author for correspondence.
Email: evmel88@gmail.com
ORCID iD: 0000-0001-5436-836X
Cand. Sci. (Med.), Neurologist
Russian Federation, MoscowAlina S. Arestova
Research Center of Neurology
Email: rizvanova.alina@gmail.com
ORCID iD: 0000-0002-9890-3552
Neurologist
Russian Federation, MoscowIrina A. Berdalina
Research Center of Neurology
Email: berdalina@neurology.ru
ORCID iD: 0009-0001-8707-180X
Statistician, Department of Training of Highly Qualified Personnel, Institute of Medical Education and Professional Development
Russian Federation, MoscowElena V. Gnedovskaya
Research Center of Neurology
Email: gnedovskaya@mail.ru
ORCID iD: 0000-0001-6026-3388
D. Sci. (Med.), Director, Institute of Medical Education and Professional Development
Russian Federation, MoscowDarya A. Grishinа
Research Center of Neurology
Email: Grishina82@gmail.com
ORCID iD: 0000-0002-7924-3405
Cand. Sci. (Med.), Head, Center for Diseases of the Peripheral Nervous System
Russian Federation, MoscowNatalia A. Suponeva
Research Center of Neurology
Email: nasu2709@mail.ru
ORCID iD: 0000-0003-3956-6362
D. Sci. (Med.), Corresponding Member of the Russian Academy of Sciences, Director, Institute of Neurorehabilitation and Restorative Technologies
Russian Federation, MoscowMichail A. Piradov
Research Center of Neurology
Email: mpi711@gmail.com
ORCID iD: 0000-0002-6338-0392
D. Sci. (Med.), Academician of the Russian Academy of Sciences, Director
Russian Federation, MoscowReferences
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