Demographics and clinical manifestations in patients with Behçet's disease: a retrospective observational study from a non-endemic country.

Abstract

Behçet's disease (BD) is a systemic vasculitis of unknown origin affecting both arterial and venous vessels, resulting in diverse clinical features. Its manifestations vary due to ethnic, geographic, and individual differences. Although the highest prevalence of BD is reported along the ancient Silk Road route, nowadays, due to population migrations, the disease can be detected worldwide. Our study aimed to evaluate the demographic and clinical characteristics of patients with BD in a cohort from a non-endemic country and compare it with other cohorts from endemic and non-endemic countries worldwide. Our retrospective observational case-series study included the data from a single Rheumatology centre. We analyzed the data from 38 patients (17 men and 21 women) with a mean age at diagnosis of 29 years ± SD 8.87 and with a mean follow-up of 12.7 years. The most common manifestations were oral (97.4%) and genital ulcerations (73.7%), skin (57.9%) and ophthalmological changes (63.1%). Other clinical manifestations, musculoskeletal (42.1%), vascular (18.4%), neurological (15.8%) and gastrointestinal (15.8%) were less common. HLA-B51 frequency was 51.5%, without significant difference between females and males (10:7 cases). All patients were treated with local therapy, while systemic glucocorticoids were given in 84.2%, followed by colchicine in 44.7%. Immunosuppressive / immunomodulator therapy (most common azathioprine) was administered in 68.4% of patients. Due to disease severity, 13.2% of patients required biological TNF- inhibitor. Although BD is most prevalent in endemic countries, the disease can be diagnosed worldwide. Due to the disease's complex pathogenesis, its varied presenting manifestations, and the lack of pathognomonic signs and symptoms, diagnosis can be delayed in non-endemic countries, leading to the untimely initiation of targeted treatment.