The importance of NLRP3 as a regulator of immune homeostasis is highlighted by the fact that mutations in the NLRP3 gene cause autoimmune diseases such as the Muckle–Wells syndrome (also known as UDA syndrome) and the familial cold autoinflammatory syndrome (urticaria episodes triggered by exposure to cold) (Hoffman et al., 2001), collectively named cryopyrin-associated periodic syndromes (Broderick et al., 2015). Here, NLRP3 is linked to familial cold autoinflammatory syndrome.