Cryopyrin-associated periodic syndrome (CAPS)
Synonyms: CAPS, NLRP3-associated autoinflammatory diseases
Overview
Cryopyrin-associated periodic syndromes (CAPS, also termed NLRP3-associated autoinflammatory diseases) are caused by uncontrolled activation of the inflammasome, leading to recurrent systemic inflammation. The clinical CAPS spectrum includes three phenotypes of increasing severity (FCAS, MWS, NOMID/CINCA), which are all caused by pathogenic variants in the NLRP3 gene and are considered to represent different degrees of severity of the same pathophysiological change.
Cryopyrin-associated periodic syndrome (CAPS)
1 Gene(s)*
NLRP3
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Last Update : 24.09.2026
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