NLRP3 and cryopyrin-associated periodic syndrome: Furthermore, gain-of-function heterozygous variants in NLRP3 are associated with a rare monogenic auto-inflammatory disease called Cryopyrin-Associated Periodic Syndrome (CAPS) and characterized by systemic inflammation and various clinical features, including mainly urticaria, arthromyalgia, buccal aphthous, and neuroinflammation with eye, hearing, and meningitis involvement [63].