AB0458 SYSTEMIC TREATMENT IN BEHÇET’S DISEASE ACCORDING TO CLINICAL PHENOTYPES. STUDY OF 111 PATIENTS
Authors: C. Alvarez Reguera, D. Martínez-López, L. Sanchez Bilbao, A. Herrero Morant, J. L. Martín-Varillas, G. Suárez Amorín, P. Setien Preciados, M. C. Mata Arnaiz, M. Á. González-Gay, R. Blanco
Journal: Annals of the Rheumatic Diseases (2020)

Background: Behçet’s disease (BD) is a multisystemic vasculitis. Different clinical phenotypes can be distinguished. Systemic corticosteroids are the most used drugs in BD. Conventional and biological immunosuppressants (IS) may also be used. Objectives: To evaluate the systemic treatment of BD according to clinical domains. Methods: Study of all consecutive 111 patients diagnosed with definitive or possible BD by expert rheumatologists in a well-defined population of Northern Spain, between 1980 and 2019. Most of them met the International Criteria for BD (ICBD) (1). Results: We studied 111 patients (62 women/49 men), mean age at diagnosis 36.8±13.2 years. After a mean follow-up of 81.4±85 months, all patients required systemic treatment (TABLE 1 -2). Biological therapy (n=28) was indicated by ocular manifestations (n=13; 46.4%) persistent, severe and refractory oral ulcers (n=10, 35.7%), neurological (n=2; 7.1%), musculoskeletal (n=2; 7.1%) or cutaneous involvement (1; 3.6%). Adalimumab and Infliximab were the biological therapy more frequently used. TABLE. CLÍNICAL PHENOTYPES Cases N (% ) COLCH COS Dosis total IS AZA MTX CYA MMF TLD APR DAP Oral ulcers 110 (99.1) 85 (77.9) 81 (73.6) 51 (46.4) 30 (27.3) 25 (22.7) 14 (12.7) 2 (1.8) 6 (5.5) 6 (5.5) 3 (2.7) Genital ulcers 69 (62.2) 56 (81.2) 51 (74) 32 (46.4) 17 (24.6) 16 (23.2) 10 (14.5) 1 (1.5) 5 (7.2) 2 (3.4) 3 (4.3) Cutaneous lesions 76 (68.5) 58 (76.3) 61 (80.3) 52 (68.4) 32 (42.1) 22 (29) 9 (11.8) 0 5 (6.6) 6 (7.9) 3 (4) Ocular manifestations 39 (35.1) 27 (69.2) 36 (92.3) 19 (48.7) 17 (43.6) 12 (30.8) 11 (28.2) 2 (5.1) 4 (10.3) 2 (5.1) 2 (5.1) Neurological involvement 20 (18) 12 (60) 15 (75) 15 (75) 3 (15) 4 (20) 3 (15) 1 (5) 0 0 1 (5) Vascular manifestations 11 (10) 8 (72.7) 9 (81.8) 5 (45.5) 3 (27.3) 3 (27.3) 1 (9.1) 0 0 0 0 Gastrointestinal involvement 4 (3.6) 2 (50) 1 (25) 1 (25) 1 (25) 0 0 0 0 0 0 TOTAL 111 85 (76.6) 85 (76.6) 51 (46) 30 (27) 25 (22.5) 14 (12.6) 2 (1.8) 6 (5.4) 6 (5.4) 3 (2.7) Conclusion: Most patients with BD required oral corticosteroids and colchicine. Almost half required conventional IS. Up to a third required biologic therapy, especially by ocular involvement. Most patients had clinical improvement. References: [1]Criteria for diagnosis of Behcet’s disease, International Study Group for Behçet’s Disease,The Lancet, Volume 335, Issue 8697, 1078 – 1080 TABLE 2. CLINICAL PHENOTYPES BT ADA IFX ETN TCZ No improvement Partial improvement Complete response Oral ulcers 28 (35.5) 22 (20) 12 (11) 3 (2.7) 2 (1.8) 22 (20) 22 (20) 66 (60) Genital ulcers 17 (24.7) 13 (18.8) 8 (11.6) 2 (2.9) 1 (1.4) 16 (23.2) 12 (17.4) 41 (59.4) Cutaneous lesions 21 (27.6) 18 (23.7) 8 (10.5) 3 (4) 2 (2.6) 8 (10.5) 19 (25) 49 (64.5) Ocular manifestations 19 (50) 16 (42.1) 9 (23.7) 1 (2.6) 2 (5.3) 0 8(21) 30 (79) Neurological involvement 7 (35) 2 (10) 4 (20) 1 (5) 0 3 (15) 5 (25) 12 (60) Vascular manifestations 4 (36.4) 3 (27.3) 2 (18.2) 1 (9.1) 1 (9.1) 2 (18.2) 4(36.4) 5 (45.5) Gastrointestinal involvement 0 0 0 0 0 1 (25) 1 (25) 2 (50) TOTAL 28 (25.2) 22 (19.8) 12 (10.8) 3 (2.7) 2 (1.8) 22 (19.8) 22 (19.8) 67 (60.4) Abbreviations: COLCH: Colchicine; OCS: Oral Corticosteroids; IS: Immunosuppressants; AZA: Azathioprine; MTX: Methotrexate; CYA: Cyclosporine A; MMF: Mycophenolate Mofetil; TLD: Talidomide; APR: Apremilast; DAP: Dapsone; BT: Biologic Therapy; ADA: Adalimumab; IFX: Infliximab; ETN: Etanercept; TCZ: Tocilizumab Disclosure of Interests: Carmen Alvarez Reguera: None declared, David Martínez-López: None declared, Lara Sanchez Bilbao: None declared, Alba Herrero Morant: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Janssen and Celgene, Speakers bureau: Pfizer and Lilly, Guillermo Suárez Amorín: None declared, Patricia Setien Preciados: None declared, M. Cristina Mata Arnaiz: None declared, Miguel Á. González-Gay Grant/research support from: AbbVie, MSD and Roche, Speakers bureau: AbbVie, MSD and Roche, Ricardo Blanco Grant/research support from: Abbvie, MSD and Roche, Consultant of: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen and MSD, Speakers bureau: Abbvie, Pfizer, Roche, Bristol-Myers, Janssen, Lilly and MSD