What Is a Periodic Fever Syndrome?

Periodic fever syndrome is a term used to describe several different conditions that can cause repeated unexplained fevers. These fevers aren’t due to known autoimmune diseases or routine infections.

Some of the conditions that can fall under the periodic fever syndromes umbrella are:

• Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome: The most common of the periodic fever disorders, this syndrome typically presents in children between the ages of 2 and 5 years old. In addition to fevers, symptoms can include mouth sores, a sore throat, and swollen lymph nodes in their neck.

• Familial Mediterranean fever (FMF): This is a genetic condition that typically affects people of Mediterranean and Middle Eastern descent. In addition to fever, people with this condition may have pain and swelling in their abdomen, chest, or joints. FMF typically appears in children younger than 10 years old.

• Tumor necrosis factor receptor-associated periodic syndrome (TRAPS): This condition may appear in childhood or in mid-adulthood. In addition to fever, there may be a painful rash, chills, and muscle pain. Eye symptoms, including redness and swelling around their eyes are also common.

• Hyperimmunoglobulin D syndrome (HIDS): Also known as mevalonate kinase-associated periodic fever syndrome, this rare genetic condition often begins in the first year of life with a sudden high fever. People may also have:
  • a skin rash
  • abdominal pain
  • vomiting
  • diarrhea
  • joint pain
  • swollen neck glands

Periodic fever syndromes are known to affect older children and adults, too. Some children have chronic period fever syndrome that continues as they age.

Adults may have the same symptoms that children do.

Repeated fevers not caused by a virus are the main symptom of these conditions.

Other common symptoms that someone may have include:

• swollen lymph nodes
• joint pain
• rashes
• swelling around their eyes
• mouth sores

Periodic fever syndromes are generally caused by autoinflammatory diseases. This means there’s a change within their immune system that causes the cells to attack their own body.

A genetic mutation is often the cause of periodic fever syndromes, and this mutation is frequently passed down through families.

Risk factors for periodic fever syndromes include:

• Family history: Having a family history of periodic fever syndromes is a risk factor.
• Ethnicity: People of Mediterranean and Middle Eastern heritage are more likely to have some of these.
• Sex: For some of the conditions, those assigned male at birth are slightly more likely to have them than those assigned female at birth.
• Age: Children are also more likely than adults to have symptoms for the first time.

Some periodic fever conditions resolve with age, but others will require treatment for the rest of an individual’s life.

The preferred treatment will depend on the specific condition involved, but medications that reduce inflammation or suppress your child’s immune system are generally used to manage periodic fever syndromes.

Medications

Medications may include steroids of colchicine.

Steroids such as prednisone can be very effective in preventing and shortening attacks for those with PFAPA syndrome.

Colchicine is often the treatment of choice for FMF because it can both reduce the severity of your child’s attacks and help prevent amyloidosis.

Biologic therapy

Biologics are medications made from living organisms and may come from a person’s own body. They help suppress their immune system or target mutated cells to keep them from harming their body. They may disarm the harmful cells or block their body’s receptors to those specific cell types.

Periodic fever syndromes aren’t often fatal. But depending on your child’s exact condition, you may have serious complications.

For those with PFAPA syndrome, there are no long-term complications associated. It often goes away on its own by the second half of your child’s life.

On the other hand, when FMF is left untreated, it can cause amyloidosis, a dangerous buildup of proteins, which can damage your child’s organs, especially their kidneys.

TRAPS may also result in amyloidosis if untreated.

The first step in a diagnosis is ruling out other causes of recurring fever such as an infection or autoimmune disease.

A doctor or healthcare professional will typically perform a physical exam for signs of swelling, rashes, and swollen glands that can indicate periodic fever syndromes.

Blood and urine tests may also be performed to look for signs of infection or inflammation. These may be performed when the individual is feeling well and when a fever is present to compare the amount of inflammation.

In some cases, genetic testing may be used to confirm the presence of an inherited condition.

Can my child have a periodic fever syndrome without getting fevers?

In order to receive a diagnosis of periodic fever syndrome, your child will need to have had repeated, otherwise unexplainable fevers. While other symptoms such as rashes and swelling can vary, a reoccurring fever is necessary for diagnosis.

Is a periodic fever syndrome contagious?

The disorders that fall under the heading of periodic fever syndromes aren’t typically contagious. They may be passed through a family in the form of a genetic mutation though.

What are some other potential causes of recurring fevers?

Infections are one of the more common causes of recurring fevers. Autoimmune conditions such as lupus may also trigger recurring fevers. In rare cases, recurring fevers may be a symptom of cancer.

Periodic fever syndromes are a group of conditions that result in repeated fevers not due to infection or autoimmune disease.

Some of these conditions are very rare, but if your child keeps getting a fever without a known cause, speak with a doctor. While periodic fever syndromes aren’t often fatal, getting your child the correct treatment is key.