A functional test might shed more light on the deleterious effect of specific variants and aid in a more straightforward diagnosis of the disease as exemplified by clinical experience in XIAP (X-linked inhibitor of apoptosis) deficiency.44 Here, we presented and validated a robust functional assay that is able to specifically stratify patients with FMF from healthy controls, as well as from patients suffering from distinct Pyrin-associated autoinflammation and related AIDs. Here, XIAP is linked to AIDS.