Cryopyrin-Associated Periodic Syndromes

**Definition:** A group of rare autosomal dominant diseases, commonly characterized by atypical URTICARIA (hives) with systemic symptoms that develop into end-organ damage. The atypical hives do not involve T-cell or autoantibody. Cryopyrin-associated periodic syndrome includes three previously distinct disorders: Familial cold autoinflammatory syndrome; Muckle-Wells Syndrome; and CINCA Syndrome, that are now considered to represent a disease continuum, all caused by NLRP3 PROTEIN mutations.

**Tree numbers:** - C16.320.382.500 - C17.800.827.368.500 - C17.800.862.945.533.500.500 - C17.800.862.945.766.500 - C20.543.480.904.533.500.500 - C20.543.480.904.766.500 - C23.550.291.500.360.500.500

**Synonyms:** - Cryopyrin Associated Periodic Syndrome - Cryopyrinopathy