Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) (Juvenile)

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a rare, genetic disorder in children. It is caused by a defective gene mutation that may be inherited from one parent leading to increased levels of inflammation throughout the body. TRAPS affects both males and females. It usually starts before age 10 but can occur any time after. The first identified cases were found in children of Irish-Scottish backgrounds, but people from all ethnic groups can be affected. Infections, injuries, stress, or strenuous exercise may trigger episodes.

What Are the Signs/Symptoms?

Children with TRAPS may have episodes of recurrent fevers, chills, severe muscle pain in the chest or arms, abdominal pain, red and swollen eyes, or a painful, red rash lasting more than one week. The rash may move from the torso to the arms and legs. Abdominal pain with nausea, diarrhea, and vomiting is common. The symptoms can be similar to another condition called Periodic Fever Aphthous Stomatitis Pharyngitis and Cervical Adenitis (PFAPA) Syndrome. Doctors may suspect TRAPS by looking at the child’s symptoms and family health history. Blood tests can show signs of inflammation during or between flares. Genetic testing confirms the diagnosis. If not treated in a timely fashion, long-lasting inflammation can lead to amyloidosis, a condition where a protein called amyloid builds up in different organs of the body. This may lead to kidney problems if left untreated.

What Are Common Treatments?

The treatment goals are to get rid of symptoms and prevent complications like amyloidosis. Corticosteroids can help with symptoms but using them for a long time can cause serious side effects. The drug etanercept (Enbrel), which blocks a protein called tumor necrosis factor, has helped some patients when given at the start of a flare and to stop attacks. Other medications like anakinra (Kineret) and canakinumab (Ilaris) that block interleukin 1, a protein involved in the inflammation of TRAPS, can also help. In rare cases, the drug tocilizumab (Actemra), which blocks interleukin-6, may be helpful.

Living with TRAPS

With treatment, patients with TRAPS can live normal lives. Patients with TRAPS should follow their treatment plans for life to avoid flares and possible amyloidosis. Patients should have regular urine and blood tests to watch for levels of amyloid protein. Even if patients feel well, they should still take their medicine. Patients and families should let their doctor know if they notice any side effects from the treatment. Patients may need psychological support to deal with the pain or emotional effects of TRAPS. While TRAPS is not curable, it is manageable.

Updated March 2025 by Maleewan Kitcharoensakkul, MD, MSCI, and reviewed by the American College of Rheumatology Committee on Communications and Marketing.

This information is provided for general education only. Individuals should consult a qualified healthcare provider for professional medical advice, diagnosis, and treatment of a medical or health condition.