Pediatric-Onset Chronic Inflammatory Demyelinating Polyneuropathy: A Multicenter Study.

Male Female Child Humans Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / therapy Immunoglobulins, Intravenous / therapeutic use Azathioprine / therapeutic use Retrospective Studies Methotrexate

Childhood Chronic inflammatory demyelinating polyneuropathy IVIg Immunotherapy Neurofascin antibodies Steroids

Journal

Pediatric neurology

ISSN: 1873-5150

Titre abrégé: Pediatr Neurol

Pays: United States

ID NLM: 8508183

Informations de publication

Date de publication:
Aug 2023

Historique:

received: 26 12 2022

revised: 31 03 2023

accepted: 22 04 2023

medline: 10 7 2023

pubmed: 28 5 2023

entrez: 28 5 2023

Statut: ppublish

Résumé

To evaluate the clinical features, demographic features, and treatment modalities of pediatric-onset chronic inflammatory demyelinating polyneuropathy (CIDP) in Turkey. The clinical data of patients between January 2010 and December 2021 were reviewed retrospectively. The patients were evaluated according to the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society Guideline on the management of CIDP (2021). In addition, patients with typical CIDP were divided into two groups according to the first-line treatment modalities (group 1: IVIg only, group 2: IVIg + steroid). The patients were further divided into two separate groups based on their magnetic resonance imaging (MRI) characteristics. A total of 43 patients, 22 (51.2%) males and 21 (48.8%) females, were included in the study. There was a significant difference between pretreatment and post-treatment modified Rankin scale (mRS) scores (P < 0.05) of all patients. First-line treatments include intravenous immunoglobulin (IVIg) (n = 19, 44.2%), IVIg + steroids (n = 20, 46.5%), steroids (n = 1, 2.3%), IVIg + steroids + plasmapheresis (n = 1, 2.3%), and IVIg + plasmapheresis (n = 1, 2.3%). Alternative agent therapy consisted of azathioprine (n = 5), rituximab (n = 1), and azathioprine + mycophenolate mofetil + methotrexate (n = 1). There was no difference between the pretreatment and post-treatment mRS scores of groups 1 and 2 (P > 0.05); however, a significant decrease was found in the mRS scores of both groups with treatment (P < 0.05). The patients with abnormal MRI had significantly higher pretreatment mRS scores compared with the group with normal MRI (P < 0.05). This multicenter study demonstrated that first-line immunotherapy modalities (IVIg vs IVIg + steroids) had equal efficacy for the treatment of patients with CIDP. We also determined that MRI features might be associated with profound clinical features, but did not affect treatment response.

Sections du résumé

BACKGROUND BACKGROUND

To evaluate the clinical features, demographic features, and treatment modalities of pediatric-onset chronic inflammatory demyelinating polyneuropathy (CIDP) in Turkey.

METHODS METHODS

The clinical data of patients between January 2010 and December 2021 were reviewed retrospectively. The patients were evaluated according to the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society Guideline on the management of CIDP (2021). In addition, patients with typical CIDP were divided into two groups according to the first-line treatment modalities (group 1: IVIg only, group 2: IVIg + steroid). The patients were further divided into two separate groups based on their magnetic resonance imaging (MRI) characteristics.

RESULTS RESULTS

A total of 43 patients, 22 (51.2%) males and 21 (48.8%) females, were included in the study. There was a significant difference between pretreatment and post-treatment modified Rankin scale (mRS) scores (P < 0.05) of all patients. First-line treatments include intravenous immunoglobulin (IVIg) (n = 19, 44.2%), IVIg + steroids (n = 20, 46.5%), steroids (n = 1, 2.3%), IVIg + steroids + plasmapheresis (n = 1, 2.3%), and IVIg + plasmapheresis (n = 1, 2.3%). Alternative agent therapy consisted of azathioprine (n = 5), rituximab (n = 1), and azathioprine + mycophenolate mofetil + methotrexate (n = 1). There was no difference between the pretreatment and post-treatment mRS scores of groups 1 and 2 (P > 0.05); however, a significant decrease was found in the mRS scores of both groups with treatment (P < 0.05). The patients with abnormal MRI had significantly higher pretreatment mRS scores compared with the group with normal MRI (P < 0.05).

CONCLUSIONS CONCLUSIONS

This multicenter study demonstrated that first-line immunotherapy modalities (IVIg vs IVIg + steroids) had equal efficacy for the treatment of patients with CIDP. We also determined that MRI features might be associated with profound clinical features, but did not affect treatment response.

Identifiants

DOI: 10.1016/j.pediatrneurol.2023.04.018 PMID: 37245275

pubmed: 37245275

pii: S0887-8994(23)00127-3

doi: 10.1016/j.pediatrneurol.2023.04.018

pii:

doi:

Substances chimiques

Immunoglobulins, Intravenous 0

Azathioprine MRK240IY2L

Methotrexate YL5FZ2Y5U1

Types de publication

Multicenter Study Journal Article

Langues

eng

Sous-ensembles de citation

Pagination

3-10