Monogenic autoinflammatory diseases include, but are not limited to Familial Mediterranean Fever (FMF), Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS), Cryopyrin-Associated Periodic Syndromes (CAPS), Hyperimmunoglobulinemia D syndrome/mevalonate kinase deficiency (HIDS/MKD), and deficiency of IL−1Ra (DIRA). The gene discussed is IL1R1; the disease is hyperimmunoglobulinemia D with periodic fever.