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Diagnosis and management of adult primary angiitis of the central nervous system: an international survey on current practices

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Abstract

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

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

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 AC2 0.30, 95% CI 0.23–0.41). For induction therapy, 81 (84%) participants reported using glucocorticoids and cyclophosphamide in > 50% of patients. After obtaining remission, 85 (89%) participants systematically introduced or maintained immunosuppressive therapy. Glucocorticoids were prescribed for a median of 12 months. Maintenance therapy with another immunosuppressant was continued for a median of 24 months. In patients who achieved remission, we explored how eight scenarios with different imaging and CSF results supported an increase in treatment. Inter-rater agreement was substantial if the patient was symptomatic (0.66, 95% CI 0.58–0.80) and moderate (0.50, 95% CI 0.45–0.60) if asymptomatic.

Conclusion

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.

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Data Availability Statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.

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Funding

AN is supported by a training grant from the Fonds de Recherche du Québec en Santé.

Author information

Authors and Affiliations

  1. Caen Normandie University, Caen, France

    Ahmad Nehme, Marion Boulanger, Achille Aouba, Emmanuel Touzé & Hubert de Boysson

  2. Department of Neurology, Centre Hospitalier Universitaire de Caen-Normandie, Avenue de la Côte de Nacre, 14000, Caen, France

    Ahmad Nehme, Marion Boulanger & Emmanuel Touzé

  3. INSERM UMR-S U1237 PhIND/BB@C, Caen, France

    Ahmad Nehme, Marion Boulanger & Emmanuel Touzé

  4. Department of Neurology, Hôpital du Sacré-cœur de Montréal, Université de Montréal, Montréal, Canada

    Sylvain Lanthier

  5. Department of Internal Medicine, Caen University Hospital, Caen, France

    Achille Aouba & Hubert de Boysson

  6. Department of Internal Medicine and Clinical Immunology, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, 75013, Paris, France

    Patrice Cacoub

  7. Sorbonne Universités, UPMC Univ Paris 06, UMR 6211, Paris, France

    Patrice Cacoub

  8. Inflammation-Immunopathology-Biotherapy Department (DHU i2B), 75005, Paris, France

    Patrice Cacoub

  9. Department of Medicine, University of Cambridge, Cambridge, UK

    David Jayne

  10. Department of Internal Medicine, Hôpital du Sacré-cœur de Montréal, Université de Montréal, Montréal, Canada

    Jean-Paul Makhzoum

  11. Division of Rheumatology, Vasculitis Clinic, Mount Sinai Hospital, University of Toronto, Toronto, Canada

    Christian Pagnoux

  12. Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France

    Maxime Rhéaume

  13. Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris, Paris, France

    Benjamin Terrier

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  1. Ahmad Nehme
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  2. Sylvain Lanthier
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Corresponding author

Correspondence to Ahmad Nehme.

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Conflicts of interest

The authors have no competing interests to declare that are relevant to the content of this article.

Ethical approval

The project was approved by the local ethics committee at the Centre Hospitalier Universitaire de Caen-Normandie (project number 2874).

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Participants gave their informed consent on the Survey Monkey ® platform.

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Nehme, A., Lanthier, S., Boulanger, M. et al. Diagnosis and management of adult primary angiitis of the central nervous system: an international survey on current practices. J Neurol 270, 1989–1998 (2023). https://doi.org/10.1007/s00415-022-11528-7

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  • DOI: https://doi.org/10.1007/s00415-022-11528-7

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