Orphanet: Cryopyrin associated periodic syndrome

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Cryopyrin-associated periodic syndrome

Disease definition

Cryopyrin associated periodic syndrome (CAPS) defines a group of autoinflammatory diseases, characterized by recurrent episodes of systemic inflammatory attacks in the absence of infection or autoimmune disease. CAPS comprises 3 disorders on a continuum of severity: severe CINCA syndrome, intermediate Muckle-Wells syndrome (MWS) and milder familial cold urticaria (FCAS) (see these terms).


Classification level: Group of disorders
  • Synonym(s):
    • CAPS
    • Cryopyrinopathy
    • NLRP3-associated systemic autoinflammatory disease
  • Prevalence: -
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: Neonatal, Infancy, Childhood, Adolescent
  • ICD-10: E85.0
  • OMIM: -
  • UMLS: C2316212
  • MeSH: D056587
  • GARD: 10927
  • MedDRA: 10068850
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