CINCA (or neonatal onset multisystem inflammatory disease, NOMID) is a rare inherited autoinflammatory disease developed due to autosomal dominant gain of function mutations in NLRP3 (NOD-, LRR- and pyrin domain-containing protein 3). The mutations lead to systemic inflammation caused by an overproduction of IL-1β and manifested by skin rash, severe arthro- and neuropathy, including contractures, aseptic meningitis, brain atrophy and mental delay [74]. Here, NLRP3 is linked to CINCA syndrome.