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
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.
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.
