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TRAPS (TNF Receptor-Associated Periodic Syndrome)

Autosomal dominant autoinflammatory disease caused by TNFRSF1A gene mutations.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is TRAPS (TNF Receptor-Associated Periodic Syndrome)?

TRAPS is an autosomal dominant autoinflammatory disorder caused by mutations in the TNFRSF1A gene encoding the type 1 TNF receptor.

The defective receptor cannot fold or be shed properly, leading to intracellular stress and uncontrolled inflammatory cytokine release.

Attacks are typically longer than other periodic fever syndromes (one to three weeks) and respond well to anti-TNF and IL-1 blockade.

Symptoms

Prolonged febrile episodes lasting one to three weeks
Migratory myalgia and overlying erythematous skin rash
Periorbital oedema and conjunctivitis
Abdominal pain mimicking peritonitis
Arthralgia and arthritis (mostly large joints)
Pleuritic chest pain and headache

Risk Factors

TNFRSF1A gene mutation (autosomal dominant inheritance)
Northern European ancestry (most common)
Family history of unexplained periodic fevers
Cysteine residue mutations (severe phenotype, amyloidosis risk)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Recurrent unexplained fever lasting more than seven days
  • Migratory rash with myalgia and periorbital swelling
  • Family history of periodic fever syndromes
  • Persistently elevated acute phase reactants between attacks (amyloidosis risk)

Treatment Methods

01
Genetic confirmation by TNFRSF1A sequencing
02
Corticosteroids for acute attacks (high response rate)
03
Etanercept (anti-TNF) as first biologic, with caution due to paradoxical flares
04
Anakinra or canakinumab (IL-1 blockade) — current preferred long-term therapy
05
Annual screening for AA amyloidosis with proteinuria and serum amyloid A measurement
06
Avoid colchicine (less effective than in FMF) and NSAIDs alone for chronic control

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Dahiliye (İç Hastalıkları) Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.