What are the symptoms?

Symptoms occur in recurrent flares, which may last from several days to a few weeks. Common features include:

• Fever
• Muscle pain (often severe and localised)
• A painful, non-itchy rash that may move across the body
• Joint pain or swelling
• Abdominal pain (which can mimic surgical conditions)
• Eye symptoms such as redness or swelling around the eyes (periorbital oedema)

Some people also experience fatigue during or after flares.

There are usually symptom-free periods between flares, although their frequency and severity vary between individuals.

While the flares themselves are intermittent, they continue throughout the person’s life and normally begin from a very early age

Triggers may include infections, physical stress, or emotional stress; but sometimes no clear trigger is identified.

TRAPS is most commonly caused by changes (variants) in the TNFRSF1A gene, which plays an important role in controlling inflammation.

These genetic changes can lead to:

• Impaired TNF receptor function
• Abnormal inflammatory signalling
• Prolonged activation of the immune system

Not all patients will have a detectable genetic variant, but this does not exclude the diagnosis.

Is TRAPS an autoimmune disease?

No - TRAPS is an autoinflammatory disease, not an autoimmune disease.

• Autoinflammatory diseases involve overactivation of the innate immune system (the body’s first-line defence)
• Autoimmune diseases involve the adaptive immune system mistakenly attacking the body’s own tissues

In TRAPS, inflammation occurs due to dysregulation of innate immune pathways, rather than targeted immune attack.

There is no single test for TRAPS. Diagnosis is based on:

• A detailed clinical history
• Blood tests showing elevated inflammatory markers (e.g. CRP, ESR, serum amyloid A) during flares
• Exclusion of other conditions (such as infection or autoimmune disease)
• Genetic testing (when available)

International guidelines (EULAR/ACR) also support diagnosis based on clinical features, particularly:

• Long-lasting fever episodes
• Migratory rash
• Periorbital oedema
• Muscle pain
• Family history

Why TRAPS diagnosis is important?

Chronic or poorly controlled inflammation can lead to complications. The most serious is amyloidosis, where inflammatory proteins build up in organs (especially the kidneys), potentially causing kidney damage or failure.

Early diagnosis and effective treatment significantly reduce this risk.

Treatment aims to control inflammation, reduce symptoms, and prevent long-term complications.

Options include:

• NSAIDs (e.g. ibuprofen, naproxen) for mild symptoms
• Corticosteroids to control acute flares (usually short-term use)
• Biologic therapies (most effective long-term), including:
  • TNF inhibitors (e.g. etanercept)
  • IL-1 inhibitors (e.g. anakinra, canakinumab)

Biologic therapies are often required for patients with frequent or severe disease and have been shown to reduce the risk of amyloidosis.

Living with TRAPS

TRAPS is a lifelong condition, and symptoms can be unpredictable. Flares may interfere with daily activities, work, or social plans.

With appropriate treatment and monitoring, many people achieve:

• Reduced flare frequency and severity
• Improved quality of life
• Lower risk of long-term complications

Ongoing follow-up is important, particularly to monitor inflammation and kidney function.