Cryopyrin-associated periodic syndrome
Engelsk navn: Cryopyrin-associated periodic syndrome
Engelske synonym: CAPS,Cryopyrinopathy,NLRP3-associated systemic autoinflammatory disease
Definisjon
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).
Fra Orphanet
ORPHA: 208650
Klassifiseringsnivå: Group of disorders
ICD-10: