Understanding Periodic Fever Syndrome:  Causes and treatment

periodic fever syndrome
Medically reviewed by Dr. Ola Tarabzuni

Overview

Periodic fever syndrome is another term for Recurrent fever syndromes, a group of disorders that cause recurring fevers. The cause of such conditions is usually autoinflammatory diseases. Additionally, most of these conditions are hereditary and arise from a gene mutation. The treatment plan depends on the type of periodic fever syndrome and the cause but often involves lifelong medication use. The article covers everything that you need to know about recurrent fever syndrome.

What causes periodic fever syndrome?

Fevers lasting several days usually accompany other symptoms, including sore throat, abdominal pain, or joint pain. These symptoms can be an indication of a periodic fever syndrome. This fever syndrome in children leads to recurrent episodes of fever, and symptoms can occur every three to four weeks. The causes of recurrent fever are linked to unregulated inflammation caused by specific hereditary conditions.

Types of periodic fevers

Periodic fever syndromes are typically rare, inherited conditions not caused by infection but by autoinflammatory conditions. The underlying cause of these cyclic fevers is unregulated inflammation in the body. A wide array of periodic fever syndrome symptoms can occur depending on the type or condition. Following are some types of this chronic fever syndrome:

  • Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome

PFAPA is the most well-known and commonly occurring disorder among these recurrent fever syndromes. The affected age group is between the ages of 2 and 5. This cyclical fever syndrome involves recurrent episodes of fever, sore throat, mouth sores and swollen lymph nodes in the neck, and the pattern is such that symptoms occur at regular intervals between episodes. Fortunately, there are no long-term complications associated with PFAPA, and treatment aims to eliminate the symptoms and focuses on returning to normal routine functioning. PFAPA may last for several years, but it tends to subside on its own during the second decade of life.

  • Familial Mediterranean Fever (FMF)

This is another type of frequent fever syndrome and is a genetic disease affecting people with a Mediterranean and Middle Eastern background, typically children under 10. Besides causing recurrent fevers, it can lead to pain and swelling in the abdomen, chest, and joints. Severe joint pain and swelling can make it hard to move around. Without prompt treatment, FMF may cause a dangerous buildup of proteins termed amyloidosis, which can eventually cause organ failure.

  • Hyperimmunoglobulin D Syndrome (HIDS)

This chronic fever is a rare genetic condition. It is also called Mevalonate Kinase-Associated Periodic Fever Syndrome. The symptoms of this pediatric periodic fever syndrome usually appear as early as the first year of life, presenting as high-grade fever, skin rash, vomiting, diarrhea, abdominal pain, joint pain, and swollen neck glands

  • Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS)

Intermittent fevers accompanied by a painful rash, muscle pain, and chills are the hallmarks of this condition. These symptoms can appear in early childhood or even around mid-adulthood. Besides the symptoms commonly occurring in periodic fever syndromes, those with TRAPS experience conjunctivitis and swelling around the eye. In some cases, amyloidosis may occur as well. 

Does your child have cyclical fevers? Talk to our doctor to find out more!

How do you treat periodic fever syndrome?

The treatment plan for periodic fever syndrome depends on the cause of these recurrent fevers. The specific diagnosis will determine the course of the treatment. The aim is to prevent episodes of fever, treat the associated symptoms, and reduce any chances of long-term side effects on the child, allowing them to carry out daily activities and go to school. Chronic periodic fever syndromes may need long-term treatment, continuing even through adulthood. Although, the condition can resolve on its own as your child grows. Some treatment options include:

  • Corticosteroids

Steroids are well-known and used to combat inflammation. PFAPA has been shown to respond well to prednisone. A single dose of this steroid can significantly reduce the duration of the attack or help resolve it. Moreover, steroids can alleviate symptoms caused by other periodic fever syndromes, but they are used cautiously to prevent any long-lasting side effects.

  • Colchicine

Colchicine is usually the treatment option for FMF as it reduces the severity of the attacks with the added benefit of helping to prevent amyloidosis. Additionally, it can be safely used for longer durations and in pregnancy.

  • Biologics

Another recent option is the use of biological therapy. Biologics are protein medications that can be injected under the skin or as an intravenous (IV) catheter, helping to effectively treat specific periodic fever syndromes associated with increased inflammatory proteins. In these cases, biologics can be more effective than corticosteroids.

How do you test for periodic fever syndrome?

To determine the cause behind recurrent fever, the pediatric rheumatologist performs a series of tests to assess your child’s condition and devise a proper treatment plan. The examination and tests comprise of the following:

  • Physical Exam

The doctor may thoroughly examine the child’s body and look for signs such as joint swelling, swollen glands, rashes, mouth sores, or swelling around the eyes. These are the symptoms that commonly occur with periodic fever syndrome, 

  • Blood and Urine Tests

These blood and urine tests help to see whether inflammation or an infection is present. The following are commonly done:

  • Blood tests, specifically white blood counts, help determine signs of infection.
  • Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP): These are blood tests to determine the level of inflammation in the body.
  • Strep culture helps diagnose strep throat.
  • Genetic Tests

Since recurrent fevers in children are also inherited, genetic testing may be necessary to help confirm a diagnosis of an inherited syndrome.

How long does periodic fever syndrome last?

Periodic fever can last for weeks, months, or years. The attacks can last for a few days as well. Depending on the type of the chronic fever, it may resolve with age or require life-long treatment. Even though some conditions may be lifelong disorders, the episodes become less frequent. Sometimes, the symptoms may last several years and eventually subside at a certain age.

Does periodic fever syndrome have long-term effects?

Spontaneous resolution for most kinds of period fever can occur, and the episodes tend to get milder and less frequent. In some cases, the symptoms resolve with time. Medication is required to deal with the symptoms and even prevent attacks in order to avoid any complications. One such complication or concerning side effect is amyloidosis.

Consult a doctor

Recurrent fever in children can be a concerning issue as the cause can be inherited diseases. It is essential to consult a doctor for diagnosis and treatment. 

If your child is experiencing a fever that comes and goes every few hours, consult your doctor at Your Doctors Online. 

Does your child have chronic fevers? Consult our doctor

FAQs about periodic fever syndrome

When does periodic fever syndrome start?

Recurrent fevers such as PFAPA usually appear in early childhood, but in rare cases, the syndrome may occur in adulthood.

How serious is periodic fever syndrome?

If the periodic fever syndrome is not treated, it may cause injury due to persistent inflammation or the chance of developing amyloidosis. 

Can stress cause periodic fever?

Acute and chronic stress can elevate body temperature and cause symptoms such as chills or aches.

Is periodic fever syndrome inherited?

Periodic fever syndromes are typically rare, inherited conditions caused by autoinflammatory conditions such as Familial Mediterranean Fever (FMF) or hyperimmunoglobulin D Syndrome (HIDS).

Your Doctors Online uses high-quality and trustworthy sources to ensure content accuracy and reliability. We rely on peer-reviewed studies, academic research institutions and medical associations to provide up-to-date and evidence-based information to the users.

  • Lachmann, Helen J. “Periodic fever syndromes.” Best practice & research Clinical rheumatology 31.4 (2017): 596-609.
  • Thomas, Kenneth Tyson, et al. “Periodic fever syndrome in children.” The Journal of pediatrics 135.1 (1999): 15-21.
  • Padeh, Shai. “Periodic fever syndromes.” Pediatric Clinics 52.2 (2005): 577-609.
  • Kastner, Daniel L. “Hereditary periodic fever syndromes.” ASH Education Program Book 2005.1 (2005): 74-81.

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