Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)

Medically Reviewed by Sabrina Felson, MD on September 07, 2022
5 min read

Fever, chills, muscle aches. These are common features of many conditions, from seasonal flu to a rare disease called tumor necrosis factor receptor-associated periodic syndrome (TRAPS). But you’d never mistake TRAPS for a common illness.

With TRAPS, fever lasts more than a week: sometimes 3 weeks, sometimes months. It’s recurrent, which means it comes back again and again throughout your life. And those muscle aches? For a lot of people, they’re more like severe muscle pain. 

TRAPS is an autoinflammatory disease that causes certain white blood cells, which normally fight infection, to attack and sometimes destroy healthy cells. It happens because a faulty gene makes your body think you have an infection when you don’t. The result: Inflammatory chemicals surge inside your body (which is how your body fights an actual infection) and you feel lousy.

TRAPS, which used to be called familial Hibernian fever, is quite rare. The odds of having it are literally around 1 in a million. Experts say only a thousand people around the world have ever been diagnosed with TRAPS.

Many rare diseases are inherited genetic disorders. That means there’s a defect in one of the genes or chromosomes you get from your parents when you’re born. 

In healthy people, this gene provides instructions to make a protein called tumor necrosis factor receptor 1 (TNFR1). That protein will bind to another protein, called tumor necrosis factor (TNF). As they join together, they send a message to cells: begin inflammation or self-destruct. This is the start of a chain of chemical reactions that help fight viruses and bacteria.

When there’s damage to TNFR1, the inflammatory process doesn’t work like it should. Scientists think that changes in the TNFR1 gene trigger excess inflammation. That’s why your immune system attacks healthy cells and causes recurrent fevers. 

TRAPS is passed down in families. When you inherit the abnormal gene from a parent, you’re at increased risk of getting sick. But you may never develop the disease. There have been some people with no family history of TRAPS who develop a new gene mutation that causes it.

Recurrent fevers are the main feature of TRAPS. Some people spike a fever every 6 weeks or so. Others can go years without a TRAPS-related episode.

Besides fever and muscle pain, symptoms include:

  • Red and swollen eyes
  • Abdominal pain
  • Nausea and vomiting
  • Diarrhea
  • Chest pain (due to inflammation around your lungs or heart)
  • A painful rash that spreads from torso to arms and legs
  • Joint pain
  • Inflammation in your mouth, throat, and digestive tract

Symptoms usually show up before age 10, but they can start in late childhood or even adulthood.

The doctor will ask about your symptoms and family medical history. One way to know for sure is a genetic test, which will determine whether you have a mutation in the TNFRSF1A gene. The doctor may also do blood tests to check for inflammation and for other types of periodic fever syndromes.

Rare diseases are often misdiagnosed. The average time from the start of symptoms to accurate diagnosis of a rare disease is around 4.8 years. With TRAPS, one reason a doctor might not quickly identify the disease is because mild cases also resemble:

  • Sore throat or strep throat: A sore throat might come with a fever. Strep throat is an infection, so a fever is likely.
  • Juvenile idiopathic arthritis: This inflammatory disease can cause joint pain and swelling, fevers, rashes, and eye irritation.
  • Familial Mediterranean fever: Similar to TRAPS, this condition usually affects people of Mediterranean and Middle Eastern descent.
  • Cervical adenitis (PFAPA) syndrome: This type of recurring fever also causes mouth sores.
  • Familial cold urticaria: This fever disorder can also cause a rash and joint pain.
  • Behçet disease: This disease causes blood vessel inflammation and symptoms such as mouth sores, eye inflammation, rashes, and sores.
  • Muckle-Wells syndrome: This genetic condition causes fever, rashes, joint pain, and chills.

As with any condition, you’re going to want to know about your outlook and treatment options. As you learn more about the disease from your doctor, here are a few things you may want to ask:

  • What medication are you prescribing?
  • What are the common side effects of this medication?
  • How do I handle pain and fever during attacks?
  • Is it safe to get routine vaccinations if I take immune-suppressing medication for TRAPS? 
  • What should I know about getting pregnant if I have TRAPS?
  • Will I have to have dialysis?
  • How do I find out if my parents or children also have genetic mutations linked to TRAPS?

Since TRAPS is such a rare disease, we don’t yet have a medication to cure it. But a few drugs have been shown to successfully treat symptoms during a flare.

  • Nonsteroidal anti-inflammatory drugs, such as ibuprofen and naproxen, can bring down fevers.
  • Corticosteroids can reduce inflammation and relieve symptoms in most people. 
  • The biologic drug etanercept (Enbrel) may lower the frequency, duration, and severity of attacks.
  • The biologic drug canakinumab (Ilaris) has been approved by the FDA to treat TRAPS in adults and children. 

You can’t always control when you’ll have a TRAPS flare, but you can reduce your likelihood of some well-known triggers, such as injury, infection, and stress. 

Your best bet is to live a healthy, balanced lifestyle. Start with these commonsense strategies:

  • Eat healthy, nutritious foods and drink alcohol in moderation.
  • Get regular, moderate exercise.
  • Make time to relax every day.
  • Avoid tobacco.
  • Don’t push yourself too hard mentally, physically, or emotionally.

Also, be sure to take any medication your doctor prescribes exactly as directed.

You’re going to have recurrent fevers and other uncomfortable symptoms when you have TRAPS. That’s what goes along with this disease. Chances are you’ll have these symptoms throughout your life.

But as you age, your fevers may not be quite as intense. Plus, having TRAPS doesn’t shorten life expectancy for most people.

There’s an exception, though. If you don’t treat it, TRAPS can lead to kidney failure. This is a result of amyloidosis which is a buildup of protein in your organs. It affects 15% to 20% of people with TRAPS. Kidney failure, or end-stage renal disease, means your kidneys have lost almost all function. To stay alive when you have kidney failure, you’ll need regular dialysis. This process removes waste and excess fluid from your body. Your doctor may recommend a kidney transplant. 

With any disease, different types of support can help in a lot of ways. Family support can ease stress and help you feel better emotionally. Health-related support groups can do the same and also be a valuable source of information on your condition. Members may help direct you to research, resources, and services.

Not sure where to start for a rare disease like TRAPS? Talk to your doctor. You may also want to check out Rareconnect.org, which has an online community for people with TRAPS and other rare diseases.