Is benign MS really benign? What a meaningful classification beyond the EDSS must take into consideration.
Benign MS
EDSS progression
Multiple sclerosis
National MS registries
Prognostic factors
Journal
Multiple sclerosis and related disorders
ISSN: 2211-0356
Titre abrégé: Mult Scler Relat Disord
Pays: Netherlands
ID NLM: 101580247
Informations de publication
Date de publication:
Nov 2020
Nov 2020
Historique:
received:
04
06
2020
revised:
26
08
2020
accepted:
02
09
2020
pubmed:
28
9
2020
medline:
15
5
2021
entrez:
27
9
2020
Statut:
ppublish
Résumé
Multiple sclerosis (MS) is a neuroinflammatory and neurodegenerative disease with an unpredictable course that has a broad clinical spectrum and progresses over time. If a person with MS (PwMS) shows overall mild to moderate disability even after a long duration of disease, the term benign MS (BMS) is used. However, there is currently no generally accepted definition of BMS. Most definitions are based on EDSS in connection with disease duration, i.e. EDSS ≤3.0 after 15 years' disease duration. The question arises whether focusing on EDSS alone is adequate for classifying the disease course taking into account that 'hidden' or 'soft' symptoms are not sufficiently covered by this instrument. The aims of the study are to assess the prevalence of BMS in one of the largest patient cohorts, to describe the prevalence of patients without disabilities and to assess the further disability progression of these patients over another 15 years. Based on data exported from the German MS Registry, PwMS with a disease duration of 15 years or more were included in the analyses. PwMS were divided into BMS (EDSS ≤3.0) or non-benign (NBMS, EDSS >3.0). Out of 31,824 PwMS included in the German MS Register, we identified 10,874 patients with a disease duration ≥15 years of whom 4,511 (42%) showed an EDSS ≤3.0 fulfilling the criterion of benign MS. In the subgroup with EDSS measured exactly at 15 years' disease duration, the proportion was 54%. This proportion decreased continuously with increasing disease duration and fell to 30% after 30 years. Female sex (hazard ratio [HR]: 0.84) was associated with BMS, while a progressive (HR: 2.09) and late disease onset (HR: 1.29) were associated with NBMS (p<0.001). With a more rigorous definition of BMS (EDSS ≤1.0, absence of disability, and active employment), only 580 (13%) of the initial BMS remained 'benign'. Our data propose an alternative definition (EDSS ≤1.0, absence from any disability, and the ability to work after 15 years of disease duration) which might truly reflect BMS.
Sections du résumé
BACKGROUND
BACKGROUND
Multiple sclerosis (MS) is a neuroinflammatory and neurodegenerative disease with an unpredictable course that has a broad clinical spectrum and progresses over time. If a person with MS (PwMS) shows overall mild to moderate disability even after a long duration of disease, the term benign MS (BMS) is used. However, there is currently no generally accepted definition of BMS. Most definitions are based on EDSS in connection with disease duration, i.e. EDSS ≤3.0 after 15 years' disease duration. The question arises whether focusing on EDSS alone is adequate for classifying the disease course taking into account that 'hidden' or 'soft' symptoms are not sufficiently covered by this instrument. The aims of the study are to assess the prevalence of BMS in one of the largest patient cohorts, to describe the prevalence of patients without disabilities and to assess the further disability progression of these patients over another 15 years.
METHODS
METHODS
Based on data exported from the German MS Registry, PwMS with a disease duration of 15 years or more were included in the analyses. PwMS were divided into BMS (EDSS ≤3.0) or non-benign (NBMS, EDSS >3.0).
RESULTS
RESULTS
Out of 31,824 PwMS included in the German MS Register, we identified 10,874 patients with a disease duration ≥15 years of whom 4,511 (42%) showed an EDSS ≤3.0 fulfilling the criterion of benign MS. In the subgroup with EDSS measured exactly at 15 years' disease duration, the proportion was 54%. This proportion decreased continuously with increasing disease duration and fell to 30% after 30 years. Female sex (hazard ratio [HR]: 0.84) was associated with BMS, while a progressive (HR: 2.09) and late disease onset (HR: 1.29) were associated with NBMS (p<0.001). With a more rigorous definition of BMS (EDSS ≤1.0, absence of disability, and active employment), only 580 (13%) of the initial BMS remained 'benign'.
CONCLUSION
CONCLUSIONS
Our data propose an alternative definition (EDSS ≤1.0, absence from any disability, and the ability to work after 15 years of disease duration) which might truly reflect BMS.
Identifiants
pubmed: 32980646
pii: S2211-0348(20)30560-5
doi: 10.1016/j.msard.2020.102485
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
102485Informations de copyright
Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.