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Hereditary Autoinflammatory Diseases

Hereditary inflammation conditions, characterized by recurrent episodes of systemic inflammation. Common symptoms include recurrent fever, rash, arthritis, fatigue, and secondary AMYLOIDOSIS. Hereditary autoinflammatory diseases are associated with mutations in genes involved in regulation of normal inflammatory process and are not caused by AUTOANTIBODIES, or antigen specific T-LYMPHOCYTES.
Also Known As:
Autoinflammation Disease, Hereditary; Autoinflammation Diseases, Hereditary; Autoinflammatory Disease, Hereditary; Autoinflammatory Diseases, Hereditary; Disease, Hereditary Autoinflammation; Disease, Hereditary Autoinflammatory; Diseases, Hereditary Autoinflammation; Diseases, Hereditary Autoinflammatory; Fever, Hereditary Recurrent; Fevers, Hereditary Recurrent; Hereditary Autoinflammation Disease; Hereditary Autoinflammatory Disease; Hereditary Recurrent Fever; Recurrent Fever, Hereditary; Recurrent Fevers, Hereditary; Hereditary Autoinflammation Diseases; Hereditary Periodic Fever Syndromes; Hereditary Recurrent Fevers
Networked: 29 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1. Inflammation
2. Familial Mediterranean Fever (Periodic Disease)
3. Fever (Fevers)
4. Cryopyrin-Associated Periodic Syndromes
5. Mevalonate Kinase Deficiency

Experts

1. Frenkel, Joost: 2 articles (05/2014 - 01/2012)
2. Goldbach-Mansky, Raphaela: 2 articles (01/2014 - 06/2013)
3. Gattorno, Marco: 2 articles (01/2012 - 11/2009)
4. Lamprecht, P: 2 articles (06/2009 - 08/2004)
5. Gross, W L: 2 articles (06/2009 - 08/2004)
6. Kastner, Daniel L: 2 articles (12/2006 - 09/2005)
7. Tas, Sander W: 1 article (01/2015)
8. Baeten, Dominique L P: 1 article (01/2015)
9. Demir, Alper: 1 article (01/2015)
10. Tak, Paul Peter: 1 article (01/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hereditary Autoinflammatory Diseases:
1. Proteins (Proteins, Gene)IBA
2. autosomal dominant familial Periodic feverIBA
3. InterleukinsIBA
01/01/2012 - "Jurg led the biochemical characterization of the inflammasome complex and demonstrated that spontaneous hyperactivation of this interleukin (IL)-1β processing machinery is the molecular basis of a spectrum of hereditary periodic fever syndromes, caused by mutated forms of the inflammasome scaffolding receptor, NLRP3. "
01/01/2013 - "Cryopyrin-associated periodic syndrome (CAPS) is a group of rare hereditary autoinflammatory diseases caused by mutations of the NLRP3 gene, and leads to excessive production of the proinflammatory cytokine, interleukin-lβ. "
06/01/2013 - "The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary "periodic fever syndromes", familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) and 6. very rare conditions presenting with autoinflammation and immunodeficiency."
4. Immunoglobulin D (IgD)IBA
5. Tumor Necrosis Factor Receptors (Tumor Necrosis Factor Receptor)IBA
6. Majeed syndromeIBA
08/01/2009 - "Among the pediatric conditions belonging to this group we can consider hereditary recurrent fevers (familial Mediterranean fever, mevalonate kinase deficiency syndrome, tumor necrosis factor receptor-associated periodic syndrome, cryopyrin-associated periodic syndromes), pyogenic disorders (PAPA syndrome, CRMO syndrome, Majeed syndrome), immune-mediated granulomatous diseases (Blau syndrome, Crohn's disease), and idiopathic febrile syndromes (systemic-onset juvenile idiopathic arthritis, PFAPA syndrome, Behçet syndrome). "
06/01/2013 - "The autoinflammatory diseases can be grouped based on clinical findings: 1. the three classic hereditary "periodic fever syndromes", familial Mediterranean Fever (FMF); TNF receptor associated periodic syndrome (TRAPS); and mevalonate kinase deficiency/hyperimmunoglobulinemia D and periodic fever syndrome (HIDS); 2. the cryopyrin associated periodic syndromes (CAPS), comprising familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and neonatal-onset multisystem inflammatory disease (NOMID) or CINCA, and; 3. pediatric granulomatous arthritis (PGA); 4. disorders presenting with skin pustules, including deficiency of interleukin 1 receptor antagonist (DIRA); Majeed syndrome; pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome; deficiency of interleukin 36 receptor antagonist (DITRA); CARD14 mediated psoriasis (CAMPS), and early-onset inflammatory bowel diseases (EO-IBD); 5. inflammatory disorders caused by mutations in proteasome components, the proteasome associated autoinflammatory syndromes (PRAAS) and 6. very rare conditions presenting with autoinflammation and immunodeficiency."
7. marenostrin (pyrin)IBA
8. Interleukin-1beta (Interleukin 1 beta)IBA
9. Pattern Recognition ReceptorsIBA
10. Proteasome Endopeptidase Complex (Proteasome)IBA