The nucleotide-binding oligomerization domain-like receptor family pyrin domain-containing proteins (NLRPs), specifically NLRP3, a cytosolic innate immune signaling receptor, have been detected at elevated levels in several inflammatory diseases, such as chronic infantile neurological cutaneous and articular (CINCA) syndrome, familial cold auto-inflammatory syndrome (FCAS), keratoendotheliitis fugax hereditaria, Muckle–Wells syndrome (MWS), neonatal onset multisystem inflammatory disease (NOMID) (2, 3), and familial Mediterranean fever (4). The gene discussed is NLRP3; the disease is CINCA syndrome.