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Descriptor English: Cryopyrin-Associated Periodic Syndromes
Descriptor Spanish: Síndromes Periódicos Asociados a Criopirina
Descriptor síndromes periódicos asociados a criopirina
Entry term(s) IOMID
NOMID
criopirinopatía
enfermedad inflamatoria multisistémica de inicio infantil
enfermedad inflamatoria multisistémica de inicio neonatal
enfermedad inflamatoria multisistémica del lactante
enfermedad inflamatoria multisistémica neonatal
enfermedad neonatal inflamatoria
enfermedad neonatal inflamatoria multisistémica
síndrome CINCA
síndrome articular cutáneo y neurológico crónico del lactante
síndrome articular, cutáneo y neurológico crónico infantil
síndrome autoinflamatorio familiar por frío
síndrome de Muckle-Wells
síndrome periódico asociado a la criopirina
urticaria familiar por frío
Scope note: Grupo de enfermedades autosómicas dominantes poco frecuentes, caracterizadas usualmente por URTICARIA (habones) atípica con síntomas sistémicos que derivan en daño al órgano diana. En las urticarias atípicas no están implicadas células T ni autoanticuerpos. El síndrome periódico asociado a criopirina incluye tres trastornos previamente identificados: el síndrome autoinflamatorio familiar por frío, el síndrome de Muckle-Wells y el síndrome CINCA, que ahora se considera que representan un continuo de enfermedades que son causadas por mutaciones de la proteína NLRP3. 
Descriptor Portuguese: Síndromes Periódicas Associadas à Criopirina
Descriptor French: Syndromes périodiques associés à la cryopyrine
Entry term(s): CINCA
CINCA Syndrome
Chronic Infantile Neurologic, Cutaneous, and Articular Syndrome
Chronic Infantile Neurological, Cutaneous, and Articular Syndrome
Chronic Neurologic Cutaneous and Articular Syndrome
Chronic Neurologic, Cutaneous, and Articular Syndrome
Chronic, Infantile, Neurological, Cutaneous, Articular Syndrome
Cold Induced Autoinflammatory Syndrome, Familial
Cold Urticaria, Familial
Cold Urticarias, Familial
Cold-Induced Autoinflammatory Syndrome, Familial
Cryopyrin Associated Periodic Syndrome
Cryopyrin Associated Periodic Syndromes
Cryopyrin-Associated Periodic Syndrome
Cryopyrinopathies
Cryopyrinopathy
FCAS1
Familial Cold Autoinflammatory Syndrome
Familial Cold Autoinflammatory Syndrome 1
Familial Cold Induced Autoinflammatory Syndrome
Familial Cold Urticaria
Familial Cold Urticarias
Familial Cold-Induced Autoinflammatory Syndrome
IOMID
IOMID Syndrome
IOMID Syndromes
Infantile Onset Multisystem Inflammatory Disease
Muckle Wells Syndrome
Muckle-Wells Syndrome
Multisystem Inflammatory Disease, Neonatal Onset
Multisystem Inflammatory Disease, Neonatal-Onset
NOMID
Neonatal Onset Multisystem Inflammatory Disease
Prieur Griscelli Syndrome
Prieur-Griscelli Syndrome
Prieur-Griscelli Syndromes
Syndrome, IOMID
Syndrome, Muckle-Wells
Syndrome, Prieur-Griscelli
Syndrome, UDA
Syndrome, Urticaria-Deafness-Amyloidosis
Syndromes, IOMID
Syndromes, Prieur-Griscelli
Syndromes, UDA
Syndromes, Urticaria-Deafness-Amyloidosis
UDA Syndrome
UDA Syndromes
Urticaria Deafness Amyloidosis Syndrome
Urticaria, Deafness and Amyloidosis
Urticaria, Familial Cold
Urticaria-Deafness-Amyloidosis Syndrome
Urticaria-Deafness-Amyloidosis Syndromes
Urticarias, Familial Cold
Tree number(s): 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
RDF Unique Identifier: https://id.nlm.nih.gov/mesh/D056587
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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Previous Indexing: Autoimmune Diseases (2001-2009)
Urticaria (1972-2009)
Public MeSH Note: 2010; see Cryopyrin-associated Periodic Syndromes, 2010
History Note: 2010; use Cryopyrin-associated Periodic Syndromes, 2010
Related: NLR Family, Pyrin Domain-Containing 3 Protein MeSH
DeCS ID: 53556
Unique ID: D056587
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 2010/01/01
Date of Entry: 2009/07/06
Revision Date: 2018/10/10
Cryopyrin-Associated Periodic Syndromes - Preferred
Concept UI M0527944
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.
Preferred term Cryopyrin-Associated Periodic Syndromes
Entry term(s) Cryopyrin Associated Periodic Syndrome
Cryopyrin Associated Periodic Syndromes
Cryopyrin-Associated Periodic Syndrome
Cryopyrinopathies
Cryopyrinopathy
Muckle-Wells Syndrome - Narrower
Concept UI M0527983
Preferred term Muckle-Wells Syndrome
Entry term(s) Muckle Wells Syndrome
Syndrome, Muckle-Wells
Syndrome, UDA
Syndrome, Urticaria-Deafness-Amyloidosis
Syndromes, UDA
Syndromes, Urticaria-Deafness-Amyloidosis
UDA Syndrome
UDA Syndromes
Urticaria Deafness Amyloidosis Syndrome
Urticaria, Deafness and Amyloidosis
Urticaria-Deafness-Amyloidosis Syndrome
Urticaria-Deafness-Amyloidosis Syndromes
Familial Cold Autoinflammatory Syndrome - Narrower
Concept UI M0527982
Preferred term Familial Cold Autoinflammatory Syndrome
Entry term(s) Cold Induced Autoinflammatory Syndrome, Familial
Cold Urticaria, Familial
Cold Urticarias, Familial
Cold-Induced Autoinflammatory Syndrome, Familial
Familial Cold Induced Autoinflammatory Syndrome
Familial Cold Urticaria
Familial Cold Urticarias
Familial Cold-Induced Autoinflammatory Syndrome
Urticaria, Familial Cold
Urticarias, Familial Cold
Chronic Infantile Neurological, Cutaneous, and Articular Syndrome - Narrower
Concept UI M0527984
Preferred term Chronic Infantile Neurological, Cutaneous, and Articular Syndrome
Entry term(s) CINCA
CINCA Syndrome
Chronic Infantile Neurologic, Cutaneous, and Articular Syndrome
Chronic Neurologic Cutaneous and Articular Syndrome
Chronic Neurologic, Cutaneous, and Articular Syndrome
Chronic, Infantile, Neurological, Cutaneous, Articular Syndrome
IOMID
IOMID Syndrome
IOMID Syndromes
Infantile Onset Multisystem Inflammatory Disease
Multisystem Inflammatory Disease, Neonatal Onset
Multisystem Inflammatory Disease, Neonatal-Onset
NOMID
Neonatal Onset Multisystem Inflammatory Disease
Prieur Griscelli Syndrome
Prieur-Griscelli Syndrome
Prieur-Griscelli Syndromes
Syndrome, IOMID
Syndrome, Prieur-Griscelli
Syndromes, IOMID
Syndromes, Prieur-Griscelli
Familial Cold Autoinflammatory Syndrome 1 - Narrower
Concept UI M000644375
Preferred term Familial Cold Autoinflammatory Syndrome 1
Entry term(s) FCAS1



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