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Polyneuritis — Clinical Guidelines

Overview

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic, immune-mediated disorder characterized by symmetrical weakness and sensory impairment in the limbs, often requiring long-term immunomodulatory therapy 7 8.

Diagnosis

Clinical Criteria: Progressive or relapsing symmetrical weakness and sensory loss in the limbs, often with areflexia 7.

Electrophysiological Tests: Nerve conduction studies typically show demyelination patterns 7.

Serum and CSF Analysis: Elevated cerebrospinal fluid protein levels without significant pleocytosis; absence of oligoclonal bands 7.

Imaging: Quantitative magnetic resonance neurography (MRN) shows promise in diagnostic evaluation 2.

Management

First-Line Treatments:

- Intravenous Immunoglobulins (IVIG): Commonly used, dosing varies but often involves 0.4 g/kg every 2-4 weeks 3. - Corticosteroids: Often used initially, though long-term efficacy is limited 3.

Adjunctive Treatments:

- Plasma Exchange (Plasmapheresis): Effective in refractory cases, particularly in pediatric patients 19. - Rituximab: Emerging as a treatment option for refractory CIDP, though efficacy data is still accumulating 5.

Special Populations

Pediatrics: CIDP can present in children with relapsing courses; plasmapheresis has shown dramatic improvement 19.

Comorbidities: Association with other conditions like membranous glomerulonephritis exists, requiring multidisciplinary management 16.

Key Recommendations

Use IVIG or corticosteroids as first-line therapy for CIDP management (Evidence: Strong 3).

Consider plasmapheresis for refractory cases, especially in pediatric patients (Evidence: Moderate 19).

Evaluate MRN for diagnostic support in CIDP, particularly to differentiate from other neuropathies (Evidence: Moderate 2).

Monitor treatment response closely using validated tools at diagnosis and throughout follow-up (Evidence: Expert opinion 3).

Evaluate for and manage comorbid conditions, as CIDP can be associated with other systemic diseases (Evidence: Moderate 16).

References

1 Schierle F, Bolmgren JL, Deleuran M, Welter O, Gebauer V. Precision dermatology 2050: AI-driven personalized monitoring and individualized treatment. Journal of the European Academy of Dermatology and Venereology : JEADV 2026. link 2 Chen H, Huang X, Bao Y, Zhao C, Lin J. The diagnostic value of quantitative assessment of MR neurography in chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis. The British journal of radiology 2023. link 3 Cintas P, Bouhour F, Cauquil C, Masingue M, Tard C, Sacconi S et al.. Current clinical management of CIDP with immunoglobulins in France: An expert opinion. Revue neurologique 2023. link 4 Saalbach A. Association of Nutrition, Obesity and Skin. Nutrients 2023. link 5 Hu J, Sun C, Lu J, Zhao C, Lin J. Efficacy of rituximab treatment in chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review and meta-analysis. Journal of neurology 2022. link 6 Melo AT, Bernardo M, Pinheiro F, Rodrigues M, Torres R, Mourão AF et al.. Assessment of the outcomes of SARS-CoV-2 infection in children and young people followed at Portuguese pediatric rheumatology clinics. ARP rheumatology 2022. link 7 Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA et al.. 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. European journal of neurology 2021. link 8 Van den Bergh PYK, van Doorn PA, Hadden RDM, Avau B, Vankrunkelsven P, Allen JA et al.. 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. Journal of the peripheral nervous system : JPNS 2021. link 9 Zimmermann P, Curtis N. Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome - A challenging diagnosis not to be missed. The Journal of infection 2016. link 10 Martinez FO, Gordon S. The evolution of our understanding of macrophages and translation of findings toward the clinic. Expert review of clinical immunology 2015. link 11 Kono H, Kimura Y, Latz E. Inflammasome activation in response to dead cells and their metabolites. Current opinion in immunology 2014. link 12 Costelloe CM, Murphy WA, Madewell JE. Imaging chronic sclerosing osteitis of the diaphysis of tubular bones. AJR. American journal of roentgenology 2009. link 13 Voils SA, Evans ME, Lane MT, Schosser RH, Rapp RP. Use of macrolides and tetracyclines for chronic inflammatory diseases. The Annals of pharmacotherapy 2005. link 14 Harsha WJ, Goco PE, Crawford JV. Remission of chronic inflammatory demyelinating polyneuropathy following adenotonsillectomy. Ear, nose, & throat journal 2003. link 15 Busby M, Donaghy M. Chronic dysimmune neuropathy. A subclassification based upon the clinical features of 102 patients. Journal of neurology 2003. link 16 Kohli A, Tandon P, Kher V. Chronic inflammatory demyelinating polyradiculoneuropathy with membranous glomerulonephritis: report of one case. Clinical neurology and neurosurgery 1992. link90115-j) 17 Kashihara K, Yabuki S. Cold-induced dysaesthesia in acute idiopathic polyneuritis. Journal of neurology 1987. link 18 Katznelson D, Gross S, Sack J. Polyneuritis following BCG re-vaccination. Postgraduate medical journal 1982. link 19 Connor RK, Ziter FA, Anstall HB. Childhood chronic relapsing polyneuropathy: dramatic improvement following plasmapheresis. Journal of clinical apheresis 1982. link

Polyneuritis