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Introduction There are several rare monogenic autoinflammatory bone disorders, e.g., deficiency of interleukin-1 receptor antagonist (DIRA, affected gene IL1RN), Majeed syndrome (affected gene LPIN2), cherubism (affected gene SH3BP2), as well as the recently described disease LIRSA/CRMO3 (affected gene IL1R1) (1–5).
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