: To evaluate the demographic and clinical characteristics of patients with Behçet's syndrome (BS), and to define their associations with the presence of major organ involvement (MOI).: Medical records of 2118 patients (964 males, 1154 females) were analysed retrospectively. MOI was defined as the presence of at least one of vascular, eye, nervous, or gastrointestinal system involvement. Univariable and multivariable binary and ordinal logistic regression analyses were applied to assess the factors that were potentially associated with MOI.: The mean ± sd age at diagnosis was 30.5 ± 9.4 years. Genital ulcer and joint involvement were more common in females (both p < 0.001), while MOI was more frequent in males (p < 0.001). Genital ulcer (p < 0.001) and vascular involvement (p = 0.006) were more common in patients with a younger age at diagnosis, while joint involvement was more common in older patients. A total of 1097 patients (51.8%) had at least one MOI, 322 (15.2%) at least two MOIs, and 48 (2.3%) at least three MOIs. Male gender, smoking history, and absence of genital ulcer were significantly associated with MOI in multivariable binary logistic regression. Multivariable ordinal regression analyses confirmed the association between MOI and male gender and smoking, but not the protective effect of genital ulcers. In both regression analyses, we found no significant effects of age, human leucocyte antigen-B51, skin involvement, or joint involvement on MOI.: Male gender and positive smoking history have a significant influence on the presence of MOI in patients with BS.

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