Periodic Fever Syndromes

Periodic Fever Syndromes

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Fevers that persist for several days and are accompanied by other symptoms such as sore throat, abdominal pain, and/or joint pain may indicate the presence of a periodic fever syndrome. Children may have recurrent episodes of fever and symptoms as often as every three to four weeks.

Duke pediatric rheumatologists are experts in identifying the many disorders that fall under the heading of periodic fever syndromes. They may recommend treatment to control symptoms so your child can return to daily activities. In rare cases, treatment may prevent potential complications from uncontrolled inflammation.

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Understanding Periodic Fever Syndromes

Periodic fever syndromes are a group of rare, often inherited conditions; there may be a family history of similar episodes. The episodes of fever are not caused by infection and they are not due to an autoimmune disease such as systemic lupus erythematosus. Periodic fever syndromes typically involve episodes of unregulated inflammation, which is why they are also known as autoinflammatory conditions.

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome
PFAPA is probably the most common disorder. It often appears in early childhood (between the ages of 2 and 5). Children have recurrent episodes of fever, mouth sores, sore throat, and swollen lymph nodes in the neck. There tend to be regular intervals between episodes. There are no long-term complications associated with PFAPA, and treatment is recommended to minimize or eliminate symptoms and allow a return to regular activities. PFAPA may last for several years, but it often goes away on its own during the second decade of life.

Familial Mediterranean Fever (FMF)
This genetic disease mostly affects people of Mediterranean and Middle Eastern descent. It causes recurrent fevers along with pain and swelling in the abdomen, chest, or joints. Painful, severe joint swelling can make it hard to walk. It typically affects children before the age of 10. Left untreated, FMF may result in a dangerous buildup of proteins, called amyloidosis, that may lead to organ failure.

Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)
This condition causes intermittent fevers plus a painful rash, chills, and muscle pain. Onset is often in childhood but may be delayed until mid-adulthood. In addition to symptoms common to other periodic fever syndromes, people with TRAPS often have associated eye symptoms including conjunctivitis and swelling around the eye. Amyloidosis may occur in some people. 

Hyperimmunoglobulin D Syndrome (HIDS)
This rare genetic condition is also known as Mevalonate Kinase-Associated Periodic Fever Syndrome. Symptoms usually start in the first year of life. It typically begins with an abrupt onset of high fever (up to 104). Associated symptoms may include skin rash, abdominal pain, vomiting, diarrhea, joint pain, and swollen neck glands.

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Diagnosis

The first step will be to rule out other causes of recurrent fever, such as infection or an autoimmune disease that may have symptoms similar to periodic fever syndrome. The pediatric rheumatologist may also perform tests in order to better diagnose your child’s condition and recommend the most appropriate treatment. 

Physical Exam

The doctor will examine your child’s body and look for signs associated with periodic fever syndrome, such as joint swelling, rashes, swollen glands, mouth sores, or swelling around the eyes. 

Blood and Urine Tests

These and other tests determine whether infection or inflammation is present. They may include a white blood cell count, throat culture, and tests for erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Tests are often performed when your child is well, and again when your child is ill, to compare the degrees of inflammation.

Genetic Tests

May help confirm a diagnosis if physical exams and lab tests indicate your child has an inherited syndrome.

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Treatments

The type of treatment your child may need will depend on the specific diagnosis. The main goal is to prevent episodes of fever and associated symptoms while minimizing the potential for side effects so your child can perform regular daily activities such as attending school. Some periodic fever syndromes require long-term treatment, including into adulthood, whereas others may resolve on their own as your child grows.

Corticosteroids

PFAPA responds well to steroids like prednisone. A single dose given at the start of an attack can significantly shorten or end the attack. Steroids can also relieve symptoms caused by other periodic fever syndromes but are used on a limited basis to prevent side effects associated with long-term use.

Colchicine

As the treatment of choice for FMF, colchicine reduces the severity of the attacks and helps prevent amyloidosis. It is safe to use for prolonged periods and in pregnancy.

Biologics

Biologic therapy is indicated in certain periodic fever syndromes. Biologics are protein medications that are given as an injection under the skin, like an insulin injection, or by infusion through an intravenous (IV) catheter. In general, periodic fever syndromes are associated with increased inflammatory proteins. Biologics address these proteins more directly than corticosteroids.

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In addition to being among the best in the country, Duke Children's Hospital & Health Center is proud to be nationally ranked in nine pediatric specialties.

Why Choose Duke

Partners with Pediatric Specialists
When necessary, our pediatric rheumatologists partner with other Duke pediatric specialists including dermatologists, nephrologists, and ophthalmologists to manage your child’s condition.

Support for Your Child and Family
Periodic fever syndromes are especially disruptive to school-age children because they cause recurrent and painful attacks can last for days or weeks. If symptoms or treatment sessions are interfering with school, our dedicated social worker can arrange accommodations to help your child keep up with their schoolwork.

National Partners in Research and Treatment
Our rheumatologists participate in a national network of doctors devoted to expanding our knowledge of these rare conditions. When appropriate, we consult with our peers to help confirm a diagnosis or share treatment discoveries.

Reviewed: 10/30/2018