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Start free with EleplanNLRP3-associated autoinflammatory disease
ORPHA:208650Clinical group
Also called CAPS · Cryopyrin associated periodic syndrome · Cryopyrinopathy · NLRP3-associated AID · NLRP3-associated autoinflammatory syndrome
What it is
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).
Key facts
- Prevalence
- 1-9 / 1 000 000 (France)
- Age of onset
- Adolescent, Childhood, Infancy, Neonatal
- Inheritance
- Autosomal dominant, Not applicable
- Classified as
- Clinical group
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Genes reported in subtypes
Orphanet records this gene on 4 more specific entries under this disorder, not on this entry itself:
A well-studied disease is usually split into subtypes, and the gene is curated on whichever one the evidence belongs to. Open a subtype to see which gene goes with it.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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