Cryopyrin-Associated Periodic Syndromes [periodické syndromy asociované s kryopyrinem]

topical
23
Terms

chronický infantilní neurologický kožní a kloubní syndrom
familiární chladová kopřivka
familiární chladová urtika
familiární chladový autoinflamatorní syndrom
FCAS1
kryopyrinopatie
Muckleův-Wellsův syndrom
multisystémové zánětlivé onemocnění se začátkem v novorozeneckém věku
NOMID
syndrom CINCA
systémové zánětlivé onemocnění se začátkem v novorozeneckém věku, charakterizované neurologickým, kožním a kloubním postižením

 

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
CINCA
CINCA Syndrome
Cold Urticaria, Familial
Cold-Induced Autoinflammatory Syndrome, Familial
Cryopyrin Associated Periodic Syndrome
Cryopyrinopathy
Familial Cold Autoinflammatory Syndrome
Familial Cold Autoinflammatory Syndrome 1
Familial Cold Urticaria
Familial Cold-Induced Autoinflammatory Syndrome
FCAS1
Infantile Onset Multisystem Inflammatory Disease
IOMID
IOMID Syndrome
Muckle-Wells Syndrome
Multisystem Inflammatory Disease, Neonatal-Onset
Neonatal Onset Multisystem Inflammatory Disease
NOMID
Prieur-Griscelli Syndrome
UDA Syndrome
Urticaria-Deafness-Amyloidosis Syndrome
Urticaria, Deafness and Amyloidosis

Persistent link   https://www.medvik.cz/link/D056587
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.

DUI
D056587 MeSH Browser
CUI
M0527944
Previous indexing
Autoimmune Diseases (2001-2009); Urticaria (1972-2009)
History note
2010; use Cryopyrin-associated Periodic Syndromes, 2010
Public note
2010; see Cryopyrin-associated Periodic Syndromes, 2010

C Diseases
C16.320.382.250 Behcet Syndrome 56
C17.800 Skin Diseases 2 796
C17.800.827.368.250 Behcet Syndrome 56
C17.800.862.945 Urticaria 298
C17.800.862.945.533 Chronic Urticaria 17
C17.800.862.945.533.500 Chronic Inducible Urticaria
C17.800.862.945.533.500.500 Cryopyrin-Associated Periodic Syndromes 23
C17.800.862.945.533.500.750 Urticaria, Solar 1
C17.800.862.945.766 Cold Urticaria
C20.543 Hypersensitivity 3 164
C20.543.480.904 Urticaria 298
C20.543.480.904.533 Chronic Urticaria 17
C20.543.480.904.533.500 Chronic Inducible Urticaria
C20.543.480.904.533.500.500 Cryopyrin-Associated Periodic Syndromes 23
C20.543.480.904.533.500.750 Urticaria, Solar 1
C20.543.480.904.766 Cold Urticaria
C23.550.291 Disease Attributes 1 207
C23.550.291.500 Chronic Disease 5 034
C23.550.291.500.360 Chronic Urticaria 17
C23.550.291.500.360.500 Chronic Inducible Urticaria
C23.550.291.500.360.500.500 Cryopyrin-Associated Periodic Syndromes 23
C23.550.291.500.360.500.750 Urticaria, Solar 1