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 spotted in children of Irish-Scottish background, but all ethnic groups are affected. Infection, trauma, 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 red painful rash lasting more than one week. The rash may move from the torso to the limbs. Abdominal pain with nausea, diarrhea, and vomiting is commonly seen. Symptoms may resemble similar conditions such as Periodic Fever Aphthous Stomatitis Pharyngitis and Cervical Adenitis (PFAPA) Syndrome. Physicians may suspect TRAPS based on physical exams and family medical history. Blood tests may show signs of inflammation during or between flares. Genetic testing confirms the diagnosis. If not treated in a timely fashion, persistent uncontrolled inflammation may lead to systemic amyloidosis. Amyloid is a protein that can build up within multiple organs of the body. This may lead to loss of kidney function without treatment.

What Are Common Treatments?

The treatment goals are to eliminate symptoms and prevent complications like amyloidosis. Corticosteroids may help ease symptoms, but long-term use has serious side effects. The drug etanercept (Enbrel), which blocks tumor necrosis factor, has been effective in some patients when given at the beginning of a flare and to prevent attacks. Medications such as anakinra (Kineret) and canakinumab (Ilaris) that block interleukin 1, a protein involved in the inflammation of TRAPS, are other effective treatments. In rare cases, the interleukin-6 blocker tocilizumab (Actemra) may be helpful.

Living with TRAPS

Patients should be able to live normal lives with available treatment. Patients with TRAPS should follow their treatment plans for life to prevent flares and possible amyloidosis. Patients should have regular urine and blood tests to watch for levels of amyloid protein. Treatments should be taken even when children feel well. Patients and families should remain aware and alert their doctor if experiencing a side effect from the medications used for treatment. Patients may need psychological support to cope or to deal with pain during TRAPS episodes. While TRAPS is not curable, it is manageable.

Updated February 2023 by David Waldburg, MD, 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 health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.

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