SAT0508 DIAGNOSIS OF BEHÇET’S DISEASE: COMPARISON OF TWO SETS OF CLASSIFICATION CRITERIA. APPLICATION IN 111 PATIENTS OF A WELL-DEFINED POPULATION
Authors: C. Alvarez Reguera, A. Herrero Morant, L. Sanchez Bilbao, D. Martínez-López, 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 systemic, chronic, relapsing vasculitis with no pathognomonic diagnostic test. The most widely used classification criteria are those of the International Study Group (ISG) for BD (1). These criteria were repeatedly found to have low sensitivity. Therefore, the International Criteria for Behçet’s Disease (ICBD) were published in 2014 (2). Objectives: To compare the ISG with ICBD diagnostic criteria for BD. Methods: The study included all consecutive 111 patients diagnosed with definitive or possible BD by expert rheumatologists. They were diagnosed at a well-defined population in Northern Spain between 1980 and 2019. The ISG and ICBD diagnostic criteria for BD were applied to and compared among all patients. Results: We studied 111 patients (62 Women/49 Men), mean age 36.8±13.2 years. BD was diagnosed in 65 (58.5%) by ISGBD criteria and in 86 (77.5%) by ICBD criteria. No significant differences were observed between both criteria (p &lt; 0.001). The overall concordance was fair (Kappa 0.3; p&lt;0.001). Sensitivity was 58.6% for ICBD criteria and 80.2% for ISG. (TABLE-1 ) Conclusion: ICBD criteria exhibit higher sensitivity than ISG criteria. Thus, the application of these new criteria can achieve a more correct and earlier diagnosis of BD. References: [1]Criteria for diagnosis of Behcet’s disease, International Study Group for Behçet’s Disease,The Lancet, Volume 335, Issue 8697, 1078 – 1080 [2]The International Criteria for Behçet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria, J Eur Acad Dermatol Venereol. 2014; 28:338-47 TABLE 1 Expert diagnosis (N=111 ) ISG criteria -(N=65 ) ICBD criteria (N=86 ) Age, mean (SD ) 36.7 (13.2) 36 (12.8) 36.7 (13) Gender, men/women, N (% ) 49/62 (44.1/55.8) 29/36 (44.6/55.4) 38/48 (44.2/55.8) Oral aphthosis 110 (99) 65 (100) 85 (100) -Recurrent (3 times/year) 91 (87.2) 61 (93.8) 74 (86) Genital aphthosis 59 (53.1) 42 (64.6) 56 (65.1) Skin manifestations 76 (68.4) 56 (86.15) 71 (70.9) -Pseudofolliculitis/ Erythema nodosum 51 (67.1)/ 27 (35.5) 38 (58.5)/ 21 (32.3) 42 (68.8)/ 22 (36.1) Ocular lesions 39 (35.1) 32 (49.2) 39 (45.3) -Anterior/ Posterior/ Panuveitis 17 (43.6);12 (30.8)/ 0 16 (50)/ 8(25)/ 7 (21.9) 17 (45.6); 0; 12 (30.8) -Retinal vasculitis 4 (10.3) 1 (3.1) 4 (10.6) Joint manifestations 76 (68.5) 43 (66.1) 58 (67.4) -Arthralgias / Arthritis 69 (92.8)/ 45 (60) 39 (90.7)/ 24 (55.8) 52 (89.6)/ 33 (56.9) Neurological manifestations 20 (18) 11 (16.9) 16 (18.6) -Peripheral / Central 11 (55)/ 14 (70) 7 (63.6)/ 7 (63.6) 12 (75)/ 10 (62.5) Vascular manifestations 9 (8.6) 7 (10.8) 10 (11.6) -Arterial/ Vein thrombosis/ Phlebitis 0/ 5 (55)/ 1 (11.1) 0/ 4 (57.1)/ 1 (14.3) 1 (12.5)/ 5 (62.5)/0 Gastrointestinal features 4 (4.5) 4 (6.1) 4 (4.6) Pathergy test positive (available data; % ) 6 (28; 21.4) 4 (19; 21) 4 (25, 16) HLA B51 positive (available data; % ) 38 (86; 44.2) 19 (47; 40.4) 28 (63; 44.4) Disclosure of Interests: Carmen Alvarez Reguera: None declared, Alba Herrero Morant: None declared, Lara Sanchez Bilbao: None declared, David Martínez-López: 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