AB1215 EPIDEMIOLOGY AND CLINICAL PHENOTYPE OF BEHÇET’S DISEASE IN A WELL-DEFINED POPULATION OF NORTHERN SPAIN
Authors: A. Herrero Morant, G. Suárez Amorín, L. Sanchez Bilbao, C. Álvarez Reguera, D. Martínez-López, J. L. Martín-Varillas, P. Setien Preciados, M. C. Mata Arnaiz, R. Demetrio-Pablo, M. Á. Gordo Vega, M. Á. González-Gay, R. Blanco
Journal: Annals of the Rheumatic Diseases (2020)

Background: Considerable epidemiological variations in prevalence of Behçet’s disease (BD) have been reported. These disparities may either reflect geographical differences, methodological artifacts, changes over time or random fluctuations. In Spain, published BD’s epidemiological studies are scarce. Objectives: To study epidemiological and clinical domains of BD in a well-defined population of Northern Spain, as well as, to compare results with other regions. Methods: We included all consecutive 111 patients, diagnosed of definitive or possible BD by expert rheumatologists between 1980 and 2019. Two Classification criteria were applied: a) International Study Group (ISG) for BD (Lancet. 1990; 335:1078-80 ), and b) International Criteria for BD (ICBD) (J Eur Acad Dermatol Venereol. 2014; 28:338-47 ). In addition, a literature review of Medline publications was carried out. Results: In our study, prevalence was higher than in most European populations regardless of the diagnostic criteria applied. Incidence was low (expert opinion: 0.021, ICBD: 0.016, ISG: 0.012). Mean age at onset (36.8±13.2) and gender distribution (55.9% females) were similar to other countries. Pathergy test was performed in 9% of patients giving low results (25.2%). Clinical domains’ frequency was in line with other regions except vascular and gastrointestinal involvement, which were lower. ( TABLE ) 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. TABLE Diagnostic criteria and study period n cases / population size Mean age at onset and sex (%females) Prevalence (over 100000) / incidence Oral / genital ulcers (%) Skin lessions/ pathergy test (%) Ocular involvement (%) Joint involvement (%) Neurobehcet/ Vascular/ Gastrointestinal involvement (%) Herrero, A et al. Southern Europe (Cantabria, Spain) Expert opinion, ISG, ICBD / 1980-2019 111 (expert opinion) / 86 (ICBD) / 65 (ISG) / 581078 36.8±13.2/ 55.9 19.1 (expert opinion), 14.8 (ICBD), 11.2 (ISG) / 0.021 (expert opinion), 0.016 (ICBD), 0.012 (ISG) 99 / 53.1 68.4 / 25. 2 35.1 68.5 18 / 10 / 4.5 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 (Seine-Saint-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. Southern Europe (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 Disclosure of Interests: Alba Herrero Morant: None declared, Guillermo Suárez Amorín: None declared, Lara Sanchez Bilbao: None declared, Carmen Álvarez Reguera: 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, Patricia Setien Preciados: None declared, M. Cristina Mata Arnaiz: None declared, Rosalía Demetrio-Pablo: None declared, Miguel Ángel Gordo Vega: 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