Autoinflammatory diseases are characterized by seemingly unprovoked inflammation. These diseases include familial Mediterranean fever; cryopyrin-associated periodic syndromes; tumor necrosis factor receptor-associated periodic syndrome; hyperimmunoglobulinemia-D syndrome; pyogenic arthritis, pyoderma gangrenosum, and acne syndrome; and Blau syndrome.Recent identification of the susceptibility genes for auto-inflammatory diseases has broadened the clinical spectrum as well as the molecular basis of these diseases. Studies have shown that activation of the interleukin-1β (IL-1β) pathway is a common mechanism in the pathogenesis of autoinflammatory diseases. A major role for the activity of a complex known as the inflammasome in the development of these diseases was established recently. New pathophysiological insights have led to the development of promising maintenance treatments designed to reduce the number and severity of the inflammatory attacks.Anakinra, a recombinant human interleukin-1 receptor antagonist, is a promising new biologic agent for the treatment of cryopyrinopathies as well other autoinflammatory diseases.