KEGG   DISEASE: Tumor necrosis factor receptor-associated periodic syndrome
Entry
H00912                      Disease                                
Name
Tumor necrosis factor receptor-associated periodic syndrome;
Familial periodic fever
Description
The tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a multisystem auto-inflammatory disorder that is inherited in an autosomal dominant manner. It is characterized by recurrent febrile attacks and localized inflammation in the absence of autoantibodies. Recurrent fever, abdominal pain, myalgia, and arthralgia are the most common manifestations of TRAPS.
Category
Immune system disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 04 Diseases of the immune system
  Autoinflammatory disorders
   4A60  Monogenic autoinflammatory syndromes
    H00912  Tumor necrosis factor receptor-associated periodic syndrome
Pathway-based classification of diseases [BR:br08402]
 Signal transduction
  nt06516  TNF signaling
   H00912  Tumor necrosis factor receptor-associated periodic syndrome
 Cellular processes
  nt06527  Necroptosis
   H00912  Tumor necrosis factor receptor-associated periodic syndrome
Pathway
hsa04668  TNF signaling pathway
hsa04064  NF-kappa B signaling pathway
hsa04217  Necroptosis
Network
nt06516 TNF signaling
nt06527 Necroptosis
Gene
TNFRSF1A [HSA:7132] [KO:K03158]
Drug
Canakinumab [DR:D09315]
Other DBs
ICD-11: 4A60.2
MeSH: C536657
OMIM: 142680
Reference
PMID:12352631
  Authors
Hull KM, Drewe E, Aksentijevich I, Singh HK, Wong K, McDermott EM, Dean J, Powell RJ, Kastner DL
  Title
The TNF receptor-associated periodic syndrome (TRAPS): emerging concepts of an autoinflammatory disorder.
  Journal
Medicine (Baltimore) 81:349-68 (2002)
DOI:10.1097/00005792-200209000-00002
Reference
PMID:16447098
  Authors
Rezaei N
  Title
TNF-receptor-associated periodic syndrome (TRAPS): an autosomal dominant multisystem disorder.
  Journal
Clin Rheumatol 25:773-7 (2006)
DOI:10.1007/s10067-005-0198-6
Reference
PMID:18408954
  Authors
Jesus AA, Oliveira JB, Aksentijevich I, Fujihira E, Carneiro-Sampaio MM, Duarte AJ, Silva CA
  Title
TNF receptor-associated periodic syndrome (TRAPS): description of a novel TNFRSF1A mutation and response to etanercept.
  Journal
Eur J Pediatr 167:1421-5 (2008)
DOI:10.1007/s00431-008-0685-2

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   OMIM (1)   
All databases (1)   

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