MWS, FCAS, and CINCA/NOMID: These systemic inflammatory diseases are autosomal dominant disorders caused by mutations in the NLRP3 (CIAS1) gene. They are characterized by intermittent episodes of fever, urticarial rash, arthralgias, and abdominal pain. In FCAS, the symptoms are precipitated by exposure to cold. In both MWS and CINCA/NOMID, progressive sensorineural hearing loss is common, while chronic meningitis, uveitis, optic disc edema, developmental delay and a characteristic deforming arthropathy (bony overgrowth) is observed only in CINCA/NOMID. Renal and systemic amyloidosis is a major cause of morbidity in MWS and FCAS. Treatment with anakinra typically results in improvement of symptoms.