A systematic review highlighted that IL-1R-targeting biologics, including anakinra—a recombinant human IL-1R1 antagonist that antagonizes bioactive IL-1α and IL-1β molecules—have been beneficial in treating rheumatoid arthritis and other autoinflammatory conditions such as periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, hyper-IgD syndrome (HIDS), cryopyrin-associated periodic syndromes (CAPS), and familial Mediterranean fever (FMF)—syndromes that are associated with mucosal manifestations [30,190,191]. Here, IL1R1 is linked to hyperimmunoglobulinemia D with periodic fever.