Eight patients were treated with anti-IL-1 therapy: anakinra in seven including three non-CAPS patients diagnosed with Schnitzler syndrome, TRAPS, and MKD, respectively (at a dose of 100 mg daily for adults and 1 mg/kg for children), and canakinumab in one (at a dose of 150 mg), and all but one (subject 10) responded completely with rapid resolution of symptoms and normalization of SAA concentration to healthy values of <4 mg/L. Here, IL1B is linked to cryopyrin-associated periodic syndrome.