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Cryopyrin-Associated Periodic Syndromes

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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.      Other names Urticarias, Familial Cold; Urticaria-Deafness-Amyloidosis Syndromes; Urticaria, Familial Cold; Urticaria Deafness Amyloidosis Syndrome; Syndromes, Urticaria-Deafness-Amyloidosis; Syndromes, Cryopyrin-Associated Periodic; Syndrome, Urticaria-Deafness-Amyloidosis; Syndrome, Muckle-Wells; Syndrome, Cryopyrin-Associated Periodic; Periodic Syndromes, Cryopyrin-Associated; Periodic Syndrome, Cryopyrin-Associated; Multisystem Inflammatory Disease, Neonatal Onset; Muckle Wells Syndrome; Familial Cold Urticarias; Cryopyrinopathies; Cryopyrin-Associated Periodic Syndrome; Cryopyrin Associated Periodic Syndromes; Cold Urticarias, Familial; Cold Urticaria, Familial; Urticaria-Deafness-Amyloidosis Syndrome
 
SubstanceCAS Registry & nameCategoriesSource
UDA syndrome  0   *Cryopyrin-Associated Periodic Syndromes.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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