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TRAPS – Tumor Necrosis Factor Receptor Associated Periodic Syndrome

TRAPS is an autoinflammatory disease characterized by unexplained periodic fever episodes or flares associated with fever and other symptoms. These flares typically last about 3 weeks, but can last from a few days to a few months. The frequency of these episodes varies greatly from person to person. The fever episodes characteristic of TRAPS can begin at any age, from infancy to late adulthood, but most people have their first episode in childhood.

Characteristics of Tumor Necrosis Factor Receptor Associated Periodic Syndrome

Symptoms

Other symptoms of TRAPS may include:

  • ​​headaches                                                                     

  • periorbital edema

  • abdominal pain                                                            

  • joint pain

  • muscle pain (myalgia)                                                

  • skin rashes

  • diarrhoea or constipation                                             

  • chills

  • nausea or vomiting                                                     

  • testicular pain

​

Males are prone to develop inguinal hernias. The most distinctive features of an attack are fever and migratory myalgia and swelling in the extremities. The overlying skin is red and tender. Skin lesions associated with TRAPS usually begin as tiny bumps (macules or papules) that spread and come together to form larger lesions (plaques).

 

Eye involvement can manifest in the form of conjunctivitis, puffiness or swelling in the skin around the eyes (periorbital edema) or uveitis. Affected individuals may also experience joint pain; and inflammation in various areas of the body including the eyes, heart muscle, certain joints, throat, or mucous membranes such as the moist lining of the mouth and digestive tract.

Flares occur either spontaneously or after minor triggers (stress, infection, exercise, etc.).

Amyloidosis involving the kidneys has been reported in 10% of patients.

Diagnosis

A diagnosis of TRAPS is made based upon a thorough clinical evaluation, identification of characteristic symptoms (e.g., long-lasting fever episodes), and blood tests for inflammatory markers. A diagnosis of TRAPS is usually confirmed by molecular genetic testing, which can identify either de novo or dominantly inherited heterozygous mutations in the TNFRSF1A gene.

During flares, laboratory tests often reveal increased indicators of inflammation: raised erythrocyte sedimentation rate (ESR), C reactive protein (CRP), fibrinogen, and serum amyloid A (SAA). Urinary protein analysis helps to monitor evolution of renal amyloidosis.

Treatment

  • IL-1 blockers (i.e. Anakinra and canakinumab)

  • Etanercept has proved to be only partially effective

  • Colchicine

  • Corticosteroids

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