With periodic occurrence of disease, lack of characteristic histopathologic finding for DM, identification of the rare NOD2 mutation and good response to small doses of prednisone (5 mg daily), Yao syndrome was diagnosed.[13] Taken together, a better understanding of the potential role of autoinflammatory components in DM could form a foundation for genetic testing for SAIDs and for a possible trial of IL-1 inhibitors in selected DM patient population. Here, IL1B is linked to dermatomyositis.