This CPB has been revised to state that rilonacept (Arcalyst) is considered: (i) medically necessary for the treatment of deficiency of interleukin-1 receptor antagonist (DIRA) when criteria are met, and (ii) experimental and investigational for the treatment of cardiovascular disorders including acute coronary syndrome, atherosclerosis, Kawasaki disease, myocardial infarction, myocarditis, and pericarditis. This CPB has been updated with the following: (i) added additional dosage and administration information, (ii) created subheaders, (iii) reorganized the policy format and subheader locations, (iv) updated the TB statement, background information, and references.