Purpose: To identify factors associated with sustained response to interleukin (IL)-1 inhibition among demographic, clinical and therapeutic data in patients with Behçet disease (BD). Methods: BD patients treated with anakinra or canakinumab were enrolled. Patients were divided into two groups according to the clinical response: group 1 included subjects showing a treatment duration of at least 52 weeks and no secondary inefﬁcacy during the ﬁrst follow-up year; the remaining patients were included in the group 2. Demographic, clinical and therapeutic data were analyzed to identify signiﬁcant differences between groups. Results: Eighteen patients were included in group 1 and 18 patients in group 2. A better response to IL-1 inhibitors was signiﬁcantly more common among patients with BD-related uveitis (p = 0.006) and patients with a longer disease duration (p = 0.03). Conclusion: IL-1 blockade is effective in BD, especially in the subset of patients presenting eye involvement and in those with long-lasting disease.