New insights into Behçet's disease in Ireland: the Midwest cohort study

OBJECTIVES: The epidemiology of Behçet's disease (BD) remains poorly understood with limited international data on disease burden, progression and treatment outcomes. The aims of this study were to determine the natural history of BD in the Midwest region of Ireland and compare our findings with those from other European and Mediterranean studies.
METHODS: We established a cohort of patients with BD in the Midwest Region of Ireland based on ISGBD and/or ICBD criteria. Longitudinal data were captured on demographic and clinical characteristics, disease activity and clinical outcomes.
RESULTS: The cohort included 24 Caucasian patients (16 women, 8 men) and one male patient with Middle Eastern ancestry, who satisfied the diagnostic criteria for BD. Based on the ISGBD criteria, the point prevalence of BD was 6.2 per 100,000 population. The most common clinical manifestation was oral aphthosis (100%) followed by genital aphthosis (92%) and skin lesions (92%), arthralgia/arthritis (40%), ocular involvement (32%), vascular thrombosis (12%) and pathergy phenomenon (8%). Only 1 patient was HLA-B*51 positive. A long-term multidisciplinary approach that included physician specialists, nurse specialists, and general practitioners was adopted for ongoing patient care.
CONCLUSIONS: The prevalence of BD in Ireland is higher than previously reported with a significant proportion experiencing laryngeal destruction. There are many similarities as well as several differences in the epidemiology of BD by country and indeed within countries. We fully advocate the need for national and international collaborative efforts in order to further understand the complex aetiology and immunopathology of BD in order to improve the clinical, physical, psychological wellbeing of patients.