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Vasculitides have been classically considered to be mostly of autoimmune origin, but the role of the innate immune system is being increasingly recognized among primary systemic vasculitides. For example, Behçet's syndrome (BS) shares more common features with autoinflammatory disorders (AIDs) than autoimmune diseases (ADs) and has recently been considered to be a polygenic AID by several authors, while others have classified it as a "mixed-pattern" disorder. This review aims to make a balance between autoinflammatory and autoimmune featur