Preferred Label : Autoinflammatory disease due to interleukin-1 receptor antagonist deficiency;
ICD-11 definition : DIRA is a monogenic autoinflammatory disease associated with mutations in the gene
coding for IL1 receptor antagonist.;
ICD-11 synonym : DIRA - [deficiency of interleukin 1 receptor antagonist]; deficiency of interleukin 1 receptor antagonist; Sterile multifocal osteomyelitis with periostitis and pustulosis;
ICD-11 acronym : DIRA;
Origin ID : 1499483400;
Currated CISMeF NLP mapping
DIRA is a monogenic autoinflammatory disease associated with mutations in the gene
coding for IL1 receptor antagonist.