Tumor necrosis factor receptor-associated periodic syndrome
Tumor necrosis factor receptor-associated periodic syndrome
Also known as:TRAPS; TNF receptor-associated periodic syndrome; China Second Rare Disease Catalog item 81
TRAPS is a rare inherited autoinflammatory disease in which TNFRSF1A variants cause recurrent long fever episodes with rash, muscle pain, abdominal pain, eye swelling, and high inflammatory markers.
Start Here
A quick guide to the next step: which department to start with, what to prepare, and what to ask.
Recurrent unexplained fever, high inflammatory markers, poor response to antibiotics, family history, rash, abdominal pain, or muscle pain should prompt rheumatology, pediatric rheumatology, or an autoinflammatory disease clinic.
TRAPS is an autoinflammatory disease. Flares can last days to weeks and may include migratory rash, deep muscle pain, abdominal pain, chest pain, joint pain, and swelling around the eyes.
Treatment aims to control attacks and chronic inflammation and prevent AA amyloidosis. IL-1 inhibitors are often used when long-term control is needed; corticosteroids or other anti-inflammatory plans may be used in selected situations.
It is usually linked to TNFRSF1A variants and often follows autosomal dominant inheritance with incomplete penetrance, so genetic counseling and family assessment are important.
Fever, abdominal pain, and rash are repeatedly treated as infection, allergy, gastroenteritis, or rheumatic fever. A fever diary, inflammatory marker pattern, and family history help shorten delay.
This page helps patients and families organize care leads. It does not replace a clinician’s diagnosis or treatment plan. For testing, medication, referrals, emergency care, and support applications, follow qualified clinicians, medical institutions, support organizations, and official sources.
Diagnosis Path
Organized around the practical patient journey: identify clues, avoid common delays, then prepare for care.
When to Suspect It
- Recurrent fever episodes lasting days to weeks, with limited antibiotic benefit and improvement between attacks.
- Fever with migratory red rash, deep muscle pain, abdominal pain, chest pain, joint pain, eye swelling, or conjunctivitis.
- Family members with recurrent fever, abdominal pain, kidney disease, proteinuria, amyloidosis, or unexplained inflammatory illness.
Common Wrong Turns
- Treating every episode as infection without reviewing duration, triggers, and well intervals.
- Only evaluating allergy, gastrointestinal disease, or infection without considering autoinflammatory disease and TNFRSF1A testing.
- Using steroids for temporary relief without monitoring SAA, urine protein, and kidney function.
Departments to Start With
- Rheumatology
- Pediatric rheumatology
- Autoinflammatory or rare immune disease clinic
- Medical genetics
Before the Visit
- Record start and end dates of fever, maximum temperature, rash photos, abdominal, chest, muscle, and joint pain, and medicine response.
- Bring flare and baseline CBC, CRP, ESR, SAA, liver and kidney function, urinalysis, and infection workup.
- Bring family history of recurrent fever, kidney disease, proteinuria, amyloidosis, early death, and genetic reports.
Tests to Ask About
- Whether an autoinflammatory gene panel should assess TNFRSF1A and other periodic fever genes.
- How CRP, ESR, SAA, and urine protein during and between attacks will guide amyloidosis risk.
- Whether IL-1 inhibition, short steroid courses, or other treatment is appropriate and how infection risk is monitored.
Questions for the Doctor
- Is my TNFRSF1A variant clearly disease-causing, and how should relatives be tested or followed?
- How can we tell ordinary infection from a TRAPS flare, and when is emergency care needed?
- Is the long-term goal no flares, normal inflammatory markers, or amyloidosis prevention?
Basic Information
Medical Notes
More complete medical explanations are kept here for discussion with clinicians.
Symptoms
TRAPS flares often last days to weeks and can include recurrent fever, chills, fatigue, migratory red or patchy rash, deep muscle pain, abdominal pain, nausea, vomiting, chest pain, joint pain, swelling around the eyes, and conjunctivitis. Some patients have low-grade inflammation between attacks.
Uncontrolled long-term inflammation can cause AA amyloidosis with proteinuria, declining kidney function, and swelling, which is a major preventable complication.
Diagnosis
Diagnosis uses the recurrent fever pattern, associated symptoms, raised inflammatory markers during flares, family history, and TNFRSF1A genetic testing. Clinicians compare flare and baseline CRP, ESR, SAA, CBC, and urine protein.
Infection, cancer, autoimmune disease, inflammatory bowel disease, and other autoinflammatory diseases such as FMF, CAPS, MKD, and PFAPA must be considered.
Treatment
Milder or infrequent attacks may be relieved with NSAIDs or short corticosteroid courses under specialist guidance, but these may not fully prevent chronic inflammation or amyloidosis risk.
Patients needing long-term control or with persistently high inflammation are often evaluated for IL-1 inhibitors. Monitoring includes infection risk, vaccines, CBC, liver and kidney function, SAA, urine protein, and quality of life.
Long-term Care
Long-term care includes a flare diary, rash photos, triggers, inflammatory markers, SAA, urinalysis and protein, kidney function, and treatment safety. Children also need growth, school, and activity planning.
A written flare plan helps avoid restarting from scratch with every fever while still staying alert for real infections.
Fertility and Family
TRAPS is usually autosomal dominant, but penetrance and severity vary. First-degree relatives can consider testing after genetic counseling; pregnancy planning should review inheritance risk and medicine safety.
When to Seek Urgent Care
Emergency care is needed for persistent high fever with poor responsiveness, dehydration, severe abdominal or chest pain, breathing trouble, altered consciousness, severe eye pain or vision change, reduced urination or swelling, or suspected serious infection during immune treatment.
Prognosis
Regular monitoring and anti-inflammatory treatment can reduce attacks; persistent inflammation and AA amyloidosis are the major threats to kidney outcome.