KEGG DISEASE: Tumor necrosis factor receptor-associated periodic syndrome

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 process
  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