Deficiency of the interleukin-1 receptor antagonist (DIRA) Forum
- 20 discussions
Deficiency of the interleukin-1 receptor antagonist (DIRA) is a very rare inherited autoinflammatory disease that usually appears at birth or in the first days or months of life. Changes in the IL1RN gene lead to uncontrolled interleukin-1 driven inflammation, causing severe skin inflammation, pustular rashes, sterile bone inflammation or osteomyelitis, and systemic flares. Without early diagnosis and treatment, it can cause serious complications and may be life-threatening.
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