AB0399 EPIDEMIOLOGY OF BEHÇET DISEASE IN A NORTHERN SPANISH HEALTH REGION

Abstract

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 periodn cases / population sizeMean age at onset and sex (%female)Prevalence (over 100000) / incidenceOral / 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- 2020120 (expert opinion) / 96 (ICBD) / 59 (ISG) / 58164137.6 ±13.8/ 48.320.6 (expert opinion), 16.5 (ICBD), 10.1 (ISG) / 0.022 (expert opinion), 0.018 (ICBD), 0.011 (ISG)94.2 / 59.263.3 / 25. 241.66510.8 / 11.6 / 6.6Calamia, K. T. et al. North America (Minnesota, USA)ISG / 1960-200513 / NR31 / 305.2 / 0.38100 / 6285 / NR624623 / 23 / NRAltenburg, A. et al. Northern Europe (Berlin, Germany)ISG and ABD classification tree / 1961-2005590 / 339134426 / 584.9 / 1 (estimated)98.5 / 63.762.5 / 33.758.15310.9 / 22.7 / 11.6Mohammad, A. et al. Northern Europe (Skane County, Sweden)ISG / 1997-201040 / 80931730.5 / 334.9 / 0.2100 / 8088 / NR53400 / 20 / NRMahr, A. et al. Southern Europe (SeineSaint-Denis County, France)ISG / 200379 / 109441227.6 / 437.1 / NR100 / 8090 / 20515910 / NR / 10Salvarani, C. et al. SouthernEurope (Reggio Emilia, Italy)ISG, 1988-200518 / 48696133 / 503.7 / 0.24100 / 78100 / NR565011 / 6 / NRAzizlerli, G. et al. Middle East (Istambul, Turkey)ISG / prevalence study101 / 23986NR / 48.542 / NR100 / 70.2Not globally reported / 69.327.7Not globally reportedNR / Not globally reported / NRDavatchi, F. et al. Middle East (Iran nationwide)Expert opinion / 1975-20187641 / NR25.6 / 44.280 / NR97.5 / 64.462.2 / 50.455.638.13.9 / 8.9 / 6.8Krause, I. et al. Middle East (Galilee, Israel)ISG / 15 years (not specific years have been reported)112 / 73700030.6 / 4715.2 / NRNR / 6841 / 44.4587011.6 / Not globally reported / NRNishiyama, M. et al. Asia (Japan nationwide)1987 JCBD / 19913316 / NR35.7 / 50.6NR / NR98.2 / 73.287.1 / 43.869.156.911 / 8.9 / 15.5JCBD: Japanese diagnostic Criteria of Behçet’s Disease; n: number of cases; NR: Not ReportedConclusion: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.