Deficiency of the interleukin-1–receptor antagonist (DIRA) is an autosomal recessive, genetic autoinflammatory syndrome resulting from mutations in IL1RN, the gene encoding the interleukin 1 receptor antagonist.

[6][7][2] DIRA displays a constellation of serious symptoms which include respiratory distress, as well as the following:[1][7] Those affected with DIRA have inherited (via autosomal recessive manner) mutations in IL1RN,[2][3] a gene that encodes a protein known as interleukin 1 receptor antagonist,[8][2] The cytogenetic location of IL1RN is 2q14.1, while its 2:113,099,364-113,134,015 are the genomic coordinates.

[3] The mechanism of deficiency of the interleukin-1–receptor antagonist affects the normal function of IL1RN gene.

[9] Those affected with deficiency of the interleukin-1–receptor antagonist can have diagnosis achieved via noting an increase of erythrocyte sedimentation rate, as well as the following:[4][3] In terms of treatment a 2013 review indicates that colchicine can be used for DIRA.

[5] Additionally there are several other management options such as anakinra, which blocks naturally occurring IL-1.