NLRP3-associated autoinflammatory disease
ORPHA:208650
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).
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- Autosomal dominant, Not applicable
- Age of onset
- Adolescent, Childhood, Infancy, Neonatal