Increasing evidence regarding the global efficacy of IL-1 inhibitors (IL-1-INH), such as Anakinra (ANA; IL-1 receptor antagonist) and Canakinumab (CAN; monoclonal anti-IL-1β antibody) has been collected from refractory systemic and articular AOSD patients (Kontzias and Efthimiou, 2012; Ortiz-Sanjuán et al., 2015). Here, IL1A is linked to adult-onset Still disease.