ABSTRACT
Background/Objectives: Neuro-Behçet's disease (NBD) is one of the most severe complications of Behçet's disease (BD). The incidence of NBD varies widely worldwide. This study aimed to estimate its current incidence in Northern Spain. Methods: This was a retrospective population-based cohort study of 120 patients in Northern Spain diagnosed with BD according to the 2013 International Criteria for BD (ICBD) between 1 January 1999 and 31 December 2019. NBD diagnoses were made according to International Consensus Recommendation (ICR) criteria. Overall, 96 patients were included, and their demographic and clinical data were collected. The incidence of NBD was estimated by age, gender, and year of diagnosis between 1999-2019. Results: NBD was diagnosed in 23 of 96 (24%) patients (15 women/8 men) (mean age: 44 ± 13.9 years). HLA-B51 was positive in 5 of 13 (38.4%) cases tested. A total of 10 (43.5%) patients had parenchymatous NBD, 10 (43.5%) had non-parenchymatous NBD, and 3 (13%) had mixed NBD. Incidence during the study period was 0.13 (95% CI, 0.11-0.26) per 100,000 people-years. There were no significant differences in gender in the incidence rate stratified by age (p > 0.05). Furthermore, there was a linear relationship with a mild decrease in age at diagnosis over time. Conclusions: Epidemiological characteristics of NBD in Northern Spain are similar to those of neighboring countries, except female gender predominance.
ABSTRACT
OBJECTIVES: The prevalence of Behçet's disease (BD) has a considerable geographical and temporal variability. Data regarding epidemiology in Spain are limited. Our study aimed to assess the epidemiology and clinical domains of BD in a population-based cohort from Northern Spain and to compare the results with other geographical areas of other countries. METHODS: We conducted a cross-sectional study of a well-defined population in Northern Spain. Cases of suspected BD between January 1980 and December 2018 were identified. The diagnosis of BD was established according to the International Study Group (ISG) for Behçet's Disease. The incidence of BD between 1999 and 2018 was estimated by sex, age, and year of diagnosis. RESULTS: Of 120 patients with probable BD, 59 patients met ISG criteria and were finally included in the study, with a male/female ratio of 0.97; mean age 49.7±14.7 years. Incidence during the period of study was 0.492 per 100,000 people, observing an increase from January 1999 to December 2018. Prevalence was 10.14 per 100,000 inhabitants in 2018. Clinical manifestations were relapsing aphthous stomatitis (100%), genital ulcers (78%), skin involvement (84.7%), joint involvement (64.4%), uveitis (55.9%), central nervous system (16.9%), vascular (10.2%), and gastrointestinal manifestations (6.8%). CONCLUSIONS: The prevalence of BD in Cantabria is higher than in other Southern European countries. This difference may reflect a combination of geographic, genetic, or methodological variations, as well as the free accessibility to the Spanish Public Health System for the entire population. Clinical phenotypes observed are similar to those described in other world regions.