Therapeutic Approaches and Predictive Factors of Relapse in Severe Ocular Involvement in Behçet's Disease: A Multicentre Retrospective Study

Audrey Hermant¹, Yann Nguyen², Fleur Cohen³, Antoine Baudet⁴, Alban DEROUX⁵, Vincent Poindron⁶, Cécile-Audrey Durel⁷, Nicolas Girszyn⁸, Noémie Le Gouellec⁹, Benedicte Rouviere¹⁰, Helder Gil¹¹, Francois Maurier¹², Nicolas Noel¹³, Loic Raffray¹⁴, Hubert De Boysson¹⁵, Benoit Faucher¹⁶, Bertrand Godeau¹⁷, Pierre Lozach¹⁸, Claude Bachmeyer¹⁹, Alice Berezne⁴, Gilles Blaison²⁰, Brice Castel²¹, Guillaume Gondran²², Matthieu Groh²³, Stéphane Giorgiutti²⁴, Jeremy Keraen²⁵, Julie Magnant²⁶, Sebastien Monnier²⁷, Philippe Blanche²⁸, Dominique Monnet²⁹, Antoine Brezin²⁹ and Benjamin Terrier³⁰, ¹Hopital Cochin, Paris, France, ²Department of Internal Medicine, Hôpital Beaujon, AP-HP, Clichy, France., Montmorency, France, ³Sorbonne Université, Paris, France, ⁴CH Annecy, Annecy, France, ⁵CHU de Grenoble, Grenoble, France, ⁶Strasbourg, Strasbourg, France, ⁷CHU Lyon, Lyon, France, ⁸Rouen, Rouen, France, ⁹CH Valenciennes, Valenciennes, France, ¹⁰CHU Brest, Brest, France, ¹¹CHU Besancon, Besançon, France, ¹²CH Metz, Metz, France, ¹³CHU Bicetre, Le Kremlin-Bicêtre, France, ¹⁴CHU la Réunion, La Réunion, France, ¹⁵CHU Caen, Caen, France, ¹⁶CHU Marseille, Marseille, France, ¹⁷CHU Henri Mondor, Créteil, France, ¹⁸CH Le Mans, Le Mans, France, ¹⁹CHU Tenon, Paris, France, ²⁰Hôpital Louis PASTEUR, Colmar, France, ²¹CH Tarbes, Tarbes, France, ²²CHU Limoges, Limoges, France, ²³National Referral Center for Hypereosinophilic Syndrome (CEREO), Hôpital Foch, Suresnes, France, ²⁴CHRU Strasbourg, Strasbourg, France, ²⁵CH Quimper, Quimper, France, ²⁶CHU Tours, Tours, France, ²⁷CH Versailles, Versailles, France, ²⁸Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, ²⁹CHU Cochin, Paris, France, ³⁰Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France

Meeting: ACR Convergence 2023

Keywords: Behçet's Syndrome, Eye Disorders, prognostic factors, TNF-blocking Antibody

Session Information

Date: Monday, November 13, 2023

Title: (1554–1578) Vasculitis – Non-ANCA-Associated & Related Disorders Poster II

Session Type: Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: The management of severe ocular involvement in Behçet’s disease (BD) is an important issue with major functional implications due to the risk of blindness. In patients with severe ocular involvement, TNF-alpha blockers have been shown to be a rapid and effective therapeutic option for controlling the disease. Once complete remission of ocular involvement has been achieved, there is a risk of relapse and potential blindness. We aimed to describe the therapeutic strategies used and to identify factors associated with ocular relapse.

Methods: This French, multicenter, retrospective study was conducted using a standardized data collection form over a 2-year period and included adults with Behçet’s disease presenting with severe ocular involvement (posterior uveitis, retinal vasculitis, macular involvement). Patients’ systemic and ocular characteristics were compared using Student’s t-tests, Chi-2 tests and Fischer tests, as appropriate. Variables associated with the risk of relapse were assessed using Cox proportional hazards models and adjusted for potential confounders.

Results: A total of 119 patients were included in this study (male/female ratio of 2.1, median age 34 (28-43) years). Severe ocular involvement was the first manifestation of BD in 101 (88%) patients. Fifty percent of patients were active smokers at the time of ocular involvement.

Severe ocular involvement consisted of posterior uveitis in 108 (91%) patients, retinal vasculitis in 86 (72%) cases, and macular involvement was found in 32 patients (34%).

Oral glucocorticoids therapy was initiated in 114 (97%) patients at a median dose of 60 mg per day (IQR 50-70), preceded by pulses of methylprednisolone in 54/110 (49%). In combination with systemic and/or topical corticosteroid therapy, 91 patients (76%) received at least one immunosuppressive and/or immunomodulatory treatment: azathioprine (n=62), TNF-alpha blockers (n=36), cyclophosphamide (n=13), mycophenolate mofetil (n=6), interferon-alpha (n=6) or methotrexate (n=5). Colchicine was prescribed in 85 patients (71%).

Characteristics and therapeutic management according to whether the presence or absence of macular involvement or the presence or absence of retinal vasculitis did not show any difference.

At the end of the first line of treatment, 99/119 patients (87%) achieved remission, and 52 of them (53%) relapsed during follow-up.

Remission and relapse rates were similar between patients with and without macular involvement and with and without retinal vasculitis. After achieving remission, the risk of relapse was significantly associated with active smoking (adjusted HR 1.92 (1.02-3.61)) and was lower in those who received prolonged glucocorticoids (adjusted HR 0.3 (0.16-0.55)), colchicine (adjusted HR 0.43 (0.22-0.83)), and anti-TNF-alpha (adjusted HR 0.5 (0.25-0.99)).

Conclusion: Among patients with Behçet’s disease and severe ocular involvement, the risk of ocular relapse was reduced by prolonged glucocorticoid therapy, systematic use of colchicine, and TNF-alpha blockers. Active smoking was associated with an increased risk of relapse, supporting the need for tobacco cessation in patients with Behçet’s disease and severe ocular involvement.

Disclosures: A. Hermant: None; Y. Nguyen: None; F. Cohen: None; A. Baudet: None; A. DEROUX: None; V. Poindron: None; C. Durel: None; N. Girszyn: None; N. Le Gouellec: None; B. Rouviere: None; H. Gil: None; F. Maurier: None; N. Noel: None; L. Raffray: None; H. De Boysson: None; B. Faucher: None; B. Godeau: None; P. Lozach: None; C. Bachmeyer: None; A. Berezne: None; G. Blaison: None; B. Castel: None; G. Gondran: None; M. Groh: AstraZeneca, 6, GSK, 6, Sanofi, 6; S. Giorgiutti: None; J. Keraen: None; J. Magnant: None; S. Monnier: None; P. Blanche: None; D. Monnet: None; A. Brezin: None; B. Terrier: AstraZeneca, 5, CSL Vifor, 2, GlaxoSmithKlein(GSK), 2.

To cite this abstract in AMA style:

Hermant A, Nguyen Y, Cohen F, Baudet A, DEROUX A, Poindron V, Durel C, Girszyn N, Le Gouellec N, Rouviere B, Gil H, Maurier F, Noel N, Raffray L, De Boysson H, Faucher B, Godeau B, Lozach P, Bachmeyer C, Berezne A, Blaison G, Castel B, Gondran G, Groh M, Giorgiutti S, Keraen J, Magnant J, Monnier S, Blanche P, Monnet D, Brezin A, Terrier B. Therapeutic Approaches and Predictive Factors of Relapse in Severe Ocular Involvement in Behçet’s Disease: A Multicentre Retrospective Study [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/therapeutic-approaches-and-predictive-factors-of-relapse-in-severe-ocular-involvement-in-behcets-disease-a-multicentre-retrospective-study/. Accessed .

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