Last update 21 Mar 2024

Deficiency of Interleukin-1 Receptor Antagonist

Basic Info

Autoinflammatory Disease due to Interleukin-1 Receptor Antagonist Deficiency, Autoinflammatory disease due to interleukin-1 receptor antagonist deficiency, DIRA
+ [22]
An autoinflammatory disease caused by mutations in the IL1RN gene, which encodes the IL1 receptor antagonist. It presents in infancy, and is characterized by systemic inflammation, pustular rash, bone pain, sterile osteitis, and periostitis.


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