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Cryopyrin-Associated Periodic Syndromes

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.
Also Known As:
CINCA; 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; Cold Urticaria, Familial; Cold-Induced Autoinflammatory Syndrome, Familial; FCAS1; Familial Cold Autoinflammatory Syndrome 1; Familial Cold-Induced Autoinflammatory Syndrome; IOMID Syndrome; Prieur-Griscelli Syndrome; UDA Syndrome; Urticaria, Deafness and Amyloidosis; Urticaria-Deafness-Amyloidosis Syndrome; Cold Induced Autoinflammatory Syndrome, Familial; Cryopyrin Associated Periodic Syndromes; Cryopyrin-Associated Periodic Syndrome; Cryopyrinopathies; Familial Cold Induced Autoinflammatory Syndrome; Familial Cold Urticarias; IOMID Syndromes; Muckle Wells Syndrome; Multisystem Inflammatory Disease, Neonatal Onset; Prieur Griscelli Syndrome; Prieur-Griscelli Syndromes; Syndrome, IOMID; Syndrome, Muckle-Wells; Syndrome, Prieur-Griscelli; Syndrome, UDA; Syndrome, Urticaria-Deafness-Amyloidosis; UDA Syndromes; Urticaria Deafness Amyloidosis Syndrome; Urticaria, Familial Cold; Urticaria-Deafness-Amyloidosis Syndromes; CINCA Syndrome; Chronic Infantile Neurological, Cutaneous, and Articular Syndrome; Cryopyrin Associated Periodic Syndrome; Cryopyrinopathy; Familial Cold Autoinflammatory Syndrome; Familial Cold Urticaria; IOMID; Infantile Onset Multisystem Inflammatory Disease; Muckle-Wells Syndrome; Multisystem Inflammatory Disease, Neonatal-Onset; NOMID; Neonatal Onset Multisystem Inflammatory Disease
Networked: 469 relevant articles (41 outcomes, 35 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Inflammation (Inflammations)
2. Cryopyrin-Associated Periodic Syndromes
3. Familial Mediterranean Fever (Periodic Disease)
4. Arthritis (Polyarthritis)
5. Urticaria (Hives)

Experts

1. Goldbach-Mansky, Raphaela: 35 articles (01/2022 - 12/2002)
2. Hoffman, Hal M: 19 articles (01/2022 - 11/2004)
3. Kuemmerle-Deschner, Jasmin B: 18 articles (10/2022 - 06/2009)
4. Rigante, Donato: 18 articles (01/2021 - 08/2010)
5. Gattorno, Marco: 17 articles (01/2022 - 09/2007)
6. Nishikomori, Ryuta: 17 articles (05/2021 - 11/2005)
7. McDermott, Michael F: 16 articles (12/2010 - 09/2002)
8. Cantarini, Luca: 14 articles (01/2020 - 08/2011)
9. Kastner, Daniel L: 13 articles (01/2022 - 12/2002)
10. Heike, Toshio: 12 articles (02/2021 - 11/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Cryopyrin-Associated Periodic Syndromes:
1. Interleukin 1 Receptor Antagonist Protein (Anakinra)FDA Link
2. canakinumabFDA Link
3. Interleukin-1 (Interleukin 1)IBA
4. InflammasomesIBA
5. CytokinesIBA
6. Tumor Necrosis Factor-alpha (Tumor Necrosis Factor)IBA
7. Interleukin-1 Receptors (Interleukin 1 Receptor)IBA
8. Monoclonal AntibodiesIBA
9. Proteins (Proteins, Gene)FDA Link
10. NLR ProteinsIBA

Therapies and Procedures

1. Therapeutics
2. Subcutaneous Injections
3. Aftercare (After-Treatment)
4. Stem Cell Transplantation
5. Injections