Diagnosis and management of adult primary angiitis of the central nervous system: an international survey on current practices.
Central nervous system vasculitis
Management
Practice
Primary angiitis of the central nervous system
Survey
Journal
Journal of neurology
ISSN: 1432-1459
Titre abrégé: J Neurol
Pays: Germany
ID NLM: 0423161
Informations de publication
Date de publication:
Apr 2023
Apr 2023
Historique:
received:
10
11
2022
accepted:
10
12
2022
revised:
09
12
2022
pubmed:
21
12
2022
medline:
22
3
2023
entrez:
20
12
2022
Statut:
ppublish
Résumé
Primary angiitis of the central nervous system (PACNS) is a rare disease, for which no validated guidelines exist. We report the findings of a survey on the clinical practice of physicians who manage adults with PACNS. An online survey was distributed through neurology, internal medicine, and rheumatology societies in Canada and Europe. Participants who were directly involved as treating physicians for at least two adult patients with PACNS were eligible for the survey. Ninety-six physicians completed the survey. Most participants were neurologists (n = 38, 40%), internists (n = 34, 35%) or rheumatologists (n = 22, 23%). Participants obtained a CNS biopsy in a median of 25% (IQR: 5-50%) of suspected PACNS cases. When determining the degree to which eight scenarios justified a CNS biopsy, participants achieved fair inter-rater agreement (Gwet's AC This survey illustrates current real-world management of PACNS and emphasizes several areas for which physicians still lack study-based evidence and/or clinical practice guidelines.
Sections du résumé
BACKGROUND
BACKGROUND
Primary angiitis of the central nervous system (PACNS) is a rare disease, for which no validated guidelines exist. We report the findings of a survey on the clinical practice of physicians who manage adults with PACNS.
METHODS
METHODS
An online survey was distributed through neurology, internal medicine, and rheumatology societies in Canada and Europe. Participants who were directly involved as treating physicians for at least two adult patients with PACNS were eligible for the survey.
RESULTS
RESULTS
Ninety-six physicians completed the survey. Most participants were neurologists (n = 38, 40%), internists (n = 34, 35%) or rheumatologists (n = 22, 23%). Participants obtained a CNS biopsy in a median of 25% (IQR: 5-50%) of suspected PACNS cases. When determining the degree to which eight scenarios justified a CNS biopsy, participants achieved fair inter-rater agreement (Gwet's AC
CONCLUSION
CONCLUSIONS
This survey illustrates current real-world management of PACNS and emphasizes several areas for which physicians still lack study-based evidence and/or clinical practice guidelines.
Identifiants
pubmed: 36538155
doi: 10.1007/s00415-022-11528-7
pii: 10.1007/s00415-022-11528-7
doi:
Substances chimiques
Immunosuppressive Agents
0
Cyclophosphamide
8N3DW7272P
Glucocorticoids
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1989-1998Subventions
Organisme : Fonds de Recherche du Québec - Santé
ID : 296911
Informations de copyright
© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.
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