OP0152 COMPARISON OF DIAGNOSTIC CRITERIA IN BEHÇET DISEASE AND SENSITIVITY IN DIAGNOSING SEVERE MANIFESTATIONS.
Authors: C. Álvarez-Reguera, A. Herrero-Morant, L. Sanchez-Bilbao, D. Martínez-López, J. L. Martín-Varillas, G. Suárez Amorín, C. Mata-Arnaiz, M. Á. González-Gay, R. Blanco
Journal: Annals of the Rheumatic Diseases (2021)

Background: Behçet disease (BD) is characterized by painful recurrent oral aphthosis genital ulcers and skin lesions. Nevertheless, the major causes of morbidity result from ocular, vascular and neurological involvement. Diagnosis of BD is usually performed according to the International Study Group (ISG ) (Lancet 1990; 335:1078-80). Recently, criteria proposed by the International Team for the Revision of the International Criteria for BD (ITR-ICBD) have demonstrated a higher sensitivity (J Eur Acad Dermatology Venereol 2014;28:338-47). Objectives: To assess a ) the concordance and differences between ISG and ICDB criteria b ) sensitivity in diagnosing severe manifestations (ocular, vascular and neurological). Methods: The study included 120 patients diagnosed with definitive or possible BD by expert rheumatologists. They were diagnosed at a well-defined population in Northern Spain between January 1980 and December 2019. The ISG and ICBD diagnostic criteria for BD were applied to all patients and compared among them. Results: 120 patients (62 men/ 58 women) were studied. Mean age at diagnosis was 37.6±13.8 years. 59 (49.2%) patients fulfilled ISG criteria and 96 (80%) ICBD criteria. Concordance between both criteria was moderate (Kappa 0.41). ICBD criteria diagnosed more patients with neurological (χ 2 =49.1, p&lt;0.01), vascular (χ 2 = 56.7, p&lt;0.01) and ocular manifestations (χ 2 =84.4 p&lt;0.01) (Figure). Figure 1. Number of patients with vascular, neurological or ocular manifestations diagnosed with BD by different criteria. Abbreviations: ITRC-ICBD: International Team for the Revision of the International Criteria for BD; ISG: International Study Group for Behçet Disease. Conclusion: ICBD criteria are more likely to diagnose BD and classify more patients with severe manifestations of the disease. References: [1]Atienza-Mateo B, et al. Rheumatology (Oxford) 2018;57(5):856-864. doi: 10.1093/rheumatology/kex480. [2]Vegas-Revenga N, et al. Am J Ophthalmol. 2019; 200:85-94. doi: 10.1016/j.ajo.2018.12.019 [3]Calvo-Río V, et al. Clin Exp Rheumatol. 2014;32(4 Suppl 84):S54-7. PMID: 25005576 [4]Santos-Gómez M, et al. Clin Exp Rheumatol. 2016;34(6 Suppl 102): S34-S40. PMID:27054359 [5]Atienza-Mateo B, et al. Arthritis Rheumatol. 2019; 71(12):2081-2089. doi: 10.1002/art.41026. [6]Martín-Varillas JL, et al. Ophthalmology. 2018;125(9):1444-1451. doi: 10.1016/j.ophtha.2018.02.020 Table 1. Main clinical features according to different diagnostic criteria. Patients characteristics, data are in n (%) Expert rheumatologists (N=120 ) ISG criteria (N=59 ) ICBD criteria (N=96 ) Age, mean years / (SD ) 38 (13.8) 35.6 (13) 37 (13) Gender, men/women, N (% ) 62/58 (52.1/47.9) 29/30 (49.1/50.8) 48/48 (50/50) Oral aphthosis, N (% ) 113 (94.2) 59 (100) 94 (97.9) Genital aphthosis, N (% ) 71 (78.5) 46 (78) 71 (74) Skin manifestations N (% ) 76 (63.3) 52 (88.1) 64 (71.6) Ocular lesions, N (% ) 54 (45) 31 (52.5) 50 (52.1) Joint manifestations, N (% ) 78 (65) 38 (64.4) 62 (64.6) Neurological manifestations, N (% ) 23 (19.2) 9 (15.2) 20 (21.1) Vascular manifestations, N (% ) 14 (11.6) 7 (11.9) 14 (14.6) Gastrointestinal features, N (% ) 8 (6.6) 3 (5.1) 5 (5.3) Disclosure of Interests: Carmen Álvarez-Reguera: None declared, Alba Herrero-Morant: None declared, Lara Sanchez-Bilbao: None declared, David Martínez-López: None declared, José Luis Martín-Varillas: None declared, Guillermo Suárez Amorín: None declared, Cristina Mata-Arnaiz: None declared, Miguel Á. González-Gay Speakers bureau: Abbvie, Pfizer, Roche, Sanofi and MSD., Grant/research support from: Abbvie, MSD, Janssen and Roche., Ricardo Blanco Speakers bureau: Abbvie, Pfizer, Bristol-Myers, Janssen, Lilly and MSD., Grant/research support from: Abbvie, MSD and Roche.