ACR Meeting Abstracts

ACR Meeting Abstracts

  • Meetings
    • ACR Convergence 2024
    • ACR Convergence 2023
    • 2023 ACR/ARP PRSYM
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • ACR Meetings

Abstract Number: 2632

Long Term Outcome Of Neuro-Behçet’s Disease

Nicolas Noel1, Remy Bernard2, Bertrand Wechsler3, Matthieu Resche-Rigon2, Du Boutin4, Jean-Charles Piette5, Aurélie Drier6, Didier Dormont6, Patrice Cacoub4 and David Saadoun7, 1APHP, Hopital Bicêtre, Service de Médecine Interne et Immunologie Clinique, Le Kremlin Bicêtre, France, 2Biostatistics, Hopital Saint-Louis, Paris, France, 3Internal Medecine, Assistance Publique-Hôpitaux de Paris, Hopital Pitié-Salpétrière, Paris, France, 4Internal Medicine, Assistance Publique-Hôpitaux de Paris, Hopital Pitié-Salpétrière, Paris, France, 5Groupe Hospitalier Pitié Salpétrière, Service de Médecine Interne, DHU i2B, Paris, France, 6Neuroradiology, Assistance Publique-Hôpitaux de Paris, Hopital Pitié-Salpétrière, Paris, France, 7DHU 2iB Internal Medicine Referal Center for Autoimmune diseases Pitie Hospital, Paris, France

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: Behcet's syndrome, Neurologic involvement, therapy and vasculitis

  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print
Session Information

Title: Vasculitis III

Session Type: Abstract Submissions (ACR)

Background/Purpose: Neurological involvement occurs in 5.3 to up to 59% of patients with Behçet’s disease (BD). Although the clinical and imaging features of neuro-Behçet’s disease (NBD) have been extensively described, few studies have reported on the long term outcome, treatment and prognosis of NBD by multivariate analyses. Moreover, no large study has focused on the impact of immunosuppressants on the outcome of NBD. In this study, we sought to report the long-term outcome of neurological involvement in patients with Behçet’s disease (BD).

Methods: Retrospective analysis of 115 patients fulfilling the International Criteria for BD [57% male with median [Q1-Q3] age of 37 [30-46] years] with neuro-Behçet’s disease (NBD) after exclusion of cerebral venous thrombosis. Factors associated with relapses of NBD, dependence and mortality were assessed by multivariate analysis. The event-free survival was calculated using Kaplan-Meier curves and a multivariate Cox proportional hazard ratio model was performed.

Results: Seventy eight (68%) patients presented with an acute onset of NBD and 37 (32%) with a progressive course. The HLA B51 allele was carried by 49% of patients. Overall, 46/115 (40%) patients had a severe initial disability status, represented by a Rankin score ≥ 3. The 5 and 7 years event-free survival rate were of 65% and 53%, respectively. In multivariate analysis, HLA-B51 positivity was independently associated with the risk of relapses of NBD (OR=4.2 [1.6-10.9]). After a median follow-up of 73 [59-102] months, 21 (25.2%) patients became dependent or died. Factors independently associated with poor outcome were a paresis at onset (OR=6.47 [1.73-24.23]) and a brainstem location of inflammatory lesions on MRI (OR=8.41 [1.03-68.43]). All the 115 patients were treated with glucocorticosteroids, including 53/115 (46.1%) with cyclophosphamide and 40/115 (34.8%) with azathioprine. A trend towards a longer event-free survival was observed in severe NBD patients (i.e. Rankin score ≥ 3 at onset) receiving intravenous cyclophosphamide compared with those treated with azathioprine (p =0.06).

Conclusion: NBD is a severe condition in which HLA-B51 carriers seems to have a worse prognosis.


Disclosure:

N. Noel,
None;

R. Bernard,
None;

B. Wechsler,
None;

M. Resche-Rigon,
None;

D. Boutin,
None;

J. C. Piette,
None;

A. Drier,
None;

D. Dormont,
None;

P. Cacoub,
None;

D. Saadoun,
None.

  • Tweet
  • Click to email a link to a friend (Opens in new window) Email
  • Click to print (Opens in new window) Print

« Back to 2013 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-outcome-of-neuro-behcets-disease/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].

Wiley

  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences

© Copyright 2025 American College of Rheumatology