At the turn of the twenty-first century, the discovery of a common genetic locus, the NLRP3 gene on chromosome 1q44, resulted in the recognition that a number of previously described clinical syndromes, including chronic infantile neurological, cutaneous and articular syndrome (CINCA), Muckle-Wells syndrome (MWS) and familial cold autoinflammatory syndrome (FCAS) were part of a wider clinical entity. Here, NLRP3 is linked to Muckle-Wells syndrome.