Behçet’s syndrome in Italy: a detailed retrospective analysis of 396 cases seen in 3 tertiary referral clinics

Behçet’s syndrome (BS) is a multisystemic disorder displaying a marked variability across different geographic areas. The\r\nmain aim of this study was to analyze demographic and clinical features of a cohort of BS patients diagnosed in three tertiary\r\nreferral centers in Italy and detect potential associations between the different manifestations. Medical records of 396 patients\r\n(218 females, 178 males) were retrospectively analyzed. Mean age at onset was 30.00 ± 18.75 years with a female-to-male\r\nratio of 1.22:1. Mucocutaneous features were the most frequent starting manifestations of BS, followed by eye inflammation.\r\nErythema nodosum (p = 0.007), arthritis/arthralgias (p = 0.0115), and central nervous system (CNS) signs (p = 0.014)\r\nwere significantly over-represented in female patients, whereas male gender was associated with lower mean age at onset\r\n(p = 0.031), higher frequency of pseudofollicular lesions, and uveitis (p = 0.00134 and p < 0.0001 respectively), particularly\r\nfor posterior segment involvement and panuveitis (p < 0.0001). Regarding the association between disease features, genital\r\nulcers were negatively associated with uveitis (p < 0.0001) and vascular involvement (p < 0.0001). Other negative associations\r\nwere detected between uveitis and gastrointestinal involvement (p = 0.008), pseudofolliculitis and CNS signs (p = 0.031),\r\nvascular involvement (p = 0.002) and erythema nodosum (p = 0.013). Logistic regression identified male gender and genital\r\nulcers, respectively, with a higher (OR 2.199 [1.397–3.461], p < 0.001) and lower risk (OR 0.157 [0.090–0.273], p < 0.0001)\r\nof developing major organ involvement. Our evaluations found that the disease had started mostly in the second and third\r\ndecade with most severe features in the male gender, and that patients presenting with mucocutaneous manifestations were\r\nless prone to develop major organ involvement.