The symptoms of neonatal-onset multisystem inflammatory disease (NOMID) usually develops during infancy, and start with symptoms such as continuous often low-grade fever, skin rash, neurological involvement, and arthropathy. Some of the possible complications include, renal failure, amyloidosis, and destructive arthropathy. Prognosis of cryopyrin-associated periodic syndrome varies according to the clinical phenotype and other factors.

• The symptoms of neonatal-onset multisystem inflammatory disease (NOMID) usually develops during infancy, and start with symptoms such as continuous often low-grade fever, skin rash, neurological involvement, and arthropathy.^[1]

• Muckle-Wells syndrome (MWS), a moderate phenotype of cryopyrin-associated periodic syndrome, manifests with a typical urticarial rash and fever.^[2]

• FCAS, the mildest form of CAPS, present in infancy with recurrent, cold-induced episodes of fever, urticaria-like skin rash, arthralgia and conjunctivitis.^[3]
• Symptoms tend to manifest in infancy, but it may present later in life, such as childhood or adolescence.^[4]
• Episodes present 1 to 3 hour after cold or cooling exposure and may take 12 to 24 hours or longer.

• Common complications of neonatal-onset multisystem inflammatory disease (NOMID) include:^[5][6]
  • Hydrocephalus and increased intracranial pressure
  • Optic nerve atrophy and progressive vision loss
  • Cognitive delay
  • Deforming arthropathy (30-40% 0f the cases)
  • Sensorineural hearing loss
  • Renal amyloidosis
• The two latter are observed more commonly among those with Muckle-Wells syndrome (MWS).^[7]

• Prognosis of cryopyrin-associated periodic syndrome varies according to the clinical phenotype and other factors.^[3]
• Factors associated with poor outcomes include:
  • Early disease onset (before the age of 6 months)
• Prognosis of NOMID is not generally good, and disabling.^[8]
• The presence of amyloidosis is associated with a particularly poor prognosis among patients with CAPS.