MeSH Browser

MeSH Heading: Cryopyrin-Associated Periodic Syndromes
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
Unique ID: D056587
RDF Unique Identifier: http://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.
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 Urticaria, Familial; Cold-Induced Autoinflammatory Syndrome, Familial; Cryopyrin Associated Periodic Syndrome; Cryopyrinopathy; FCAS1; Familial Cold Autoinflammatory Syndrome; Familial Cold Autoinflammatory Syndrome 1; Familial Cold Urticaria; Familial Cold-Induced Autoinflammatory Syndrome; IOMID; IOMID Syndrome; Infantile Onset Multisystem Inflammatory Disease; Muckle-Wells Syndrome; Multisystem Inflammatory Disease, Neonatal-Onset; NOMID; Neonatal Onset Multisystem Inflammatory Disease; Prieur-Griscelli Syndrome; UDA Syndrome; Urticaria, Deafness and Amyloidosis; Urticaria-Deafness-Amyloidosis Syndrome
Previous Indexing: Autoimmune Diseases (2001-2009); Urticaria (1972-2009)
See Also: NLR Family, Pyrin Domain-Containing 3 Protein
Public MeSH Note: 2010; see Cryopyrin-associated Periodic Syndromes, 2010
History Note: 2010; use Cryopyrin-associated Periodic Syndromes, 2010
Date Established: 2010/01/01
Date of Entry: 2009/07/06
Revision Date: 2023/04/05

Allowable Qualifiers: blood (BL)

cerebrospinal fluid (CF)

chemically induced (CI)

classification (CL)

complications (CO)

diagnosis (DI)

diagnostic imaging (DG)

diet therapy (DH)

drug therapy (DT)

economics (EC)

embryology (EM)

enzymology (EN)

epidemiology (EP)

ethnology (EH)

etiology (ET)

genetics (GE)

history (HI)

immunology (IM)

metabolism (ME)

microbiology (MI)

mortality (MO)

nursing (NU)

parasitology (PS)

pathology (PA)

physiopathology (PP)

prevention & control (PC)

psychology (PX)

radiotherapy (RT)

rehabilitation (RH)

surgery (SU)

therapy (TH)

urine (UR)

veterinary (VE)

virology (VI)

Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
  - Hereditary Autoinflammatory Diseases [C16.320.382]

Skin and Connective Tissue Diseases [C17]
- Skin Diseases [C17.800]
  - Skin Diseases, Genetic [C17.800.827]
    - Hereditary Autoinflammatory Diseases [C17.800.827.368]
      - Behcet Syndrome [C17.800.827.368.250]
      - Cryopyrin-Associated Periodic Syndromes [C17.800.827.368.500]

Skin and Connective Tissue Diseases [C17]
- Skin Diseases [C17.800]
  - Skin Diseases, Vascular [C17.800.862]
    - Urticaria [C17.800.862.945]
      - Chronic Urticaria [C17.800.862.945.533]
        
        Chronic Inducible Urticaria [C17.800.862.945.533.500]
        
        Cryopyrin-Associated Periodic Syndromes [C17.800.862.945.533.500.500]
        
        Urticaria, Solar [C17.800.862.945.533.500.750]

Skin and Connective Tissue Diseases [C17]
- Skin Diseases [C17.800]
  - Skin Diseases, Vascular [C17.800.862]
    - Urticaria [C17.800.862.945]
      - Cold Urticaria [C17.800.862.945.766]
        
        Cryopyrin-Associated Periodic Syndromes [C17.800.862.945.766.500]

Immune System Diseases [C20]
- Hypersensitivity [C20.543]
  - Hypersensitivity, Immediate [C20.543.480]
    - Urticaria [C20.543.480.904]
      - Chronic Urticaria [C20.543.480.904.533]
        
        Chronic Inducible Urticaria [C20.543.480.904.533.500]
        
        Cryopyrin-Associated Periodic Syndromes [C20.543.480.904.533.500.500]
        
        Urticaria, Solar [C20.543.480.904.533.500.750]

Immune System Diseases [C20]
- Hypersensitivity [C20.543]
  - Hypersensitivity, Immediate [C20.543.480]
    - Urticaria [C20.543.480.904]
      - Cold Urticaria [C20.543.480.904.766]
        
        Cryopyrin-Associated Periodic Syndromes [C20.543.480.904.766.500]

Pathological Conditions, Signs and Symptoms [C23]
- Pathologic Processes [C23.550]
  - Disease Attributes [C23.550.291]
    - Chronic Disease [C23.550.291.500]
      - Chronic Urticaria [C23.550.291.500.360]
        
        Chronic Inducible Urticaria [C23.550.291.500.360.500]
        
        Cryopyrin-Associated Periodic Syndromes [C23.550.291.500.360.500.500]
        
        Urticaria, Solar [C23.550.291.500.360.500.750]

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.
Terms: Cryopyrin-Associated Periodic Syndromes Preferred Term
Term UI T769283
Date04/13/2010
LexicalTag NON
ThesaurusID NLM (2011); Cryopyrin Associated Periodic Syndrome
Term UI T001090107
Date10/15/2020
LexicalTag NON
ThesaurusID NLM (2022); Cryopyrinopathy
Term UI T732274
Date12/23/2008
LexicalTag NON
ThesaurusID NLM (2010)

Familial Cold Autoinflammatory Syndrome Narrower

Muckle-Wells Syndrome Narrower

Chronic Infantile Neurological, Cutaneous, and Articular Syndrome Narrower

Familial Cold Autoinflammatory Syndrome 1 Narrower

Cryopyrin-Associated Periodic Syndromes MeSH Descriptor Data 2025