Cryopyrin-Associated Periodic Syndrome (CAPS) is caused by NLRP3 activating mutations that cause activation of the cryopyrin inflammasome and release of inflammatory cytokines including IL-1 Selective inhibition of NLRP3 could be beneficial, as NLRP3 inflammasomes are primarily involved in the inflammation process in these patients.
CAPS is a rare life-long auto-inflammatory condition, and is classified under orphan diseases. The chronic inflammation due to IL-1beta release in CAPS patients leads to urticaria-like rash, fever, arthralgia, and increased risk of amyloidosis. CAPS patients also experience multiple neurological complications like sensorineural hearing loss, migraine, headache, aseptic meningitis and myalgia. Bone deformities and neurological impairment have been reported in Neonatal Onset Multisystem Inflammatory Disease (NOMID), the most severe form of CAPS.
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