AB0399 EPIDEMIOLOGY OF BEHÇET DISEASE IN A NORTHERN SPANISH HEALTH REGION
Authors: L. Sanchez-Bilbao, G. Suárez-Amorín, C. Álvarez-Reguera, A. Herrero-Morant, D. Martínez-López, J. L. Martín-Varillas, M. C. Mata Arnaiz, R. Demetrio-Pablo, M. A. González-Gay, R. Blanco
Journal: Annals of the Rheumatic Diseases (2021)

Background: Behçet disease (BD) is a systemic and potentially severe disease. Its incidence varies widely worldwide. In Spain, published BD’s epidemiological studies are scarce. Objectives: In a well-defined Northern Spanish population-based cohort, the aim of this study was a ) to estimate epidemiological variations, b ) clinical domains and c ) to compare our results with other regions. Methods: Study of unselected all consecutive patients diagnosed with definitive or possible BD by expert rheumatologists between 1980 and 2020 in our health region. Two classification criteria were applied: a ) International Study Group (ISG) for BD [1] , and b ) International Criteria for BD (ICBD) [2] . In addition, a literature review of Medline publications was carried out. Results: In this study, from a total of 120 patients diagnosed with BD by expert opinion (58 women/62 men), 96 met ICBD and 59 ISG criteria. Mean age of the cohort at diagnosis was 37.6±13.8 years similar to other countries, as well as sex distribution. Prevalence was higher than in most European populations regardless the diagnostic criteria applied: expert opinion (20.6), ICBD (16.5) or ISG (10.1) (TABLE 1). Incidence was lower (expert opinion: 0.022, ICBD: 0.018, ISG: 0.011). Clinical domains’ frequency was in line with other regions except vascular and gastrointestinal involvement, which were lower. TABLE 1. Diagnostic criteria and study period n cases / population size Mean age at onset and sex (%female ) Prevalence (over 100000) / incidence Oral / genital ulcers (% ) Skin lessions/ pathergy test (% ) Ocular involve ment (% ) Joint involve ment (% ) Neurobehcet/ Vascular/ Gastrointestinalinvolvement (% ) Sánchez. L et al. Southern Europe (Cantabria, Spain ) Expert opinion, ISG, ICBD / 1980- 2020 120 (expert opinion) / 96 (ICBD) / 59 (ISG) / 581641 37.6 ±13.8/ 48.3 20.6 (expert opinion), 16.5 (ICBD), 10.1 (ISG) / 0.022 (expert opinion), 0.018 (ICBD), 0.011 (ISG) 94.2 / 59.2 63.3 / 25. 2 41.6 65 10.8 / 11.6 / 6.6 Calamia, K. T. et al. North America (Minnesota, USA ) ISG / 1960-2005 13 / NR 31 / 30 5.2 / 0.38 100 / 62 85 / NR 62 46 23 / 23 / NR Altenburg, A. et al. Northern Europe (Berlin, Germany ) ISG and ABD classification tree / 1961-2005 590 / 3391344 26 / 58 4.9 / 1 (estimated) 98.5 / 63.7 62.5 / 33.7 58.1 53 10.9 / 22.7 / 11.6 Mohammad, A. et al. Northern Europe (Skane County, Sweden ) ISG / 1997-2010 40 / 809317 30.5 / 33 4.9 / 0.2 100 / 80 88 / NR 53 40 0 / 20 / NR Mahr, A. et al. Southern Europe (SeineSaint-Denis County, France ) ISG / 2003 79 / 1094412 27.6 / 43 7.1 / NR 100 / 80 90 / 20 51 59 10 / NR / 10 Salvarani, C. et al. SouthernEurope (Reggio Emilia, Italy ) ISG, 1988-2005 18 / 486961 33 / 50 3.7 / 0.24 100 / 78 100 / NR 56 50 11 / 6 / NR Azizlerli, G. et al. Middle East (Istambul, Turkey ) ISG / prevalence study 101 / 23986 NR / 48.5 42 / NR 100 / 70.2 Not globally reported / 69.3 27.7 Not globally reported NR / Not globally reported / NR Davatchi, F. et al. Middle East (Iran nationwide ) Expert opinion / 1975-2018 7641 / NR 25.6 / 44.2 80 / NR 97.5 / 64.4 62.2 / 50.4 55.6 38.1 3.9 / 8.9 / 6.8 Krause, I. et al. Middle East (Galilee, Israel ) ISG / 15 years (not specific years have been reported) 112 / 737000 30.6 / 47 15.2 / NR NR / 68 41 / 44.4 58 70 11.6 / Not globally reported / NR Nishiyama, M. et al. Asia (Japan nationwide ) 1987 JCBD / 1991 3316 / NR 35.7 / 50.6 NR / NR 98.2 / 73.2 87.1 / 43.8 69.1 56.9 11 / 8.9 / 15.5 JCBD: Japanese diagnostic Criteria of Behçet’s Disease; n : number of cases; NR: Not Reported Conclusion: BD’s prevalence in Northern Spain is higher than in most European populations. These differences likely reflect a combination of true geographic variation, methodological artifacts as well as the easy access to Public Health System and its efficiency. In contrast, clinical phenotypes are similar to other regions. References: [1] Lancet. 1990; 335:1078-80 [2] J Eur Acad Dermatol Venereol. 2014; 28:338-47 Disclosure of Interests: Lara Sanchez-Bilbao: None declared, Guillermo Suárez-Amorín: None declared, Carmen Álvarez-Reguera: None declared, Alba Herrero-Morant: None declared, David Martínez-López: None declared, José Luis Martín-Varillas: None declared, M. Cristina Mata Arnaiz: None declared, Rosalía Demetrio-Pablo: None declared, Miguel A González-Gay Speakers bureau: AbbVie, Pfizer, Roche, Sanofi, Celgene and MSD., Grant/research support from: AbbVie, MSD, Jansen and Roche, Ricardo Blanco Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, Lilly and MSD., Grant/research support from: AbbVie, MSD and Roche.