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Concept information

Preferred term

Cryopyrin-Associated Periodic Syndromes  

Type

  • Topical Descriptor

Entry terms

  • Cryopyrin Associated Periodic Syndrome
  • Cryopyrinopathies
  • Cryopyrinopathy

Scope note

  • 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.

History note

  • 2010; use Cryopyrin-associated Periodic Syndromes, 2010

In other languages

  • Finnish

  • kryopyriiniin liittyvä ajoittainen oireyhtymä
  • kryopyriiniin liittyvä periodinen oireyhtymä
  • kryopyriiniin liittyvät periodiset oireyhtymät
  • kryopyrinopatia
  • kryopyrinopatiat
  • Swedish

  • CINCA-syndrom
  • Familjärt autoinflammatoriskt köldsyndrom
  • Kroniskt infantilt neurologiskt hud- och ledsyndrom
  • Mucle-Wells syndrom
  • Prieur – Griscellis syndrom

URI

http://www.yso.fi/onto/mesh/D056587

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