Periodic Fever Syndromes

What is a periodic fever syndrome?

Periodic fever syndromes refer to a group of rare conditions that cause children to have regular fevers alternating with periods of wellness. The fevers are not caused by a virus or bacteria and are usually accompanied by other symptoms. These conditions are rare and can be caused by a genetic mutation that is passed down from parents to their children.

Periodic fever syndromes include:

• Familial Mediterranean fever (FMF)
• Periodic fever aphthous-stomatitis, pharyngitis, adenitis (PFAPA) 
• Tumor necrosis factor receptor associated periodic syndrome (TRAPS)
• Hyperimmunoglobulin D syndrome (HIDS), also called mevalonate kinase associated periodic fever syndrome
• Neonatal onset multisystem inflammatory disease (NOMID)
• Muckle-Wells syndrome
• Familial cold auto inflammatory syndrome

Periodic fever syndromes generally occur in childhood or adolescence, often before the age of 10. They vary in severity and duration and are not contagious.

Physicians at the Pediatric Rheumatology Program are experienced in the diagnosis and treatment of these rare conditions, providing compassionate care and expert treatment for your child.

What are the symptoms of periodic fever syndrome?

Episodes of fever are the main symptom of these conditions.

In addition, your child may experience some of the following symptoms:

• Rash
• Abdominal pain
• Mouth sores
• Joint pain or swelling
• Headache
• Diarrhea

How are periodic fever syndromes diagnosed?

A pediatric rheumatologist will make a diagnosis based on several different factors:

• Medical history
• Symptoms
• Child’s ethnicity (certain syndromes are prevalent in specific areas of the world)
• Blood tests
• Genetic test

Symptoms of periodic fever syndrome are similar to those of other conditions, so it’s important to see a physician who has experience with diagnosing these diseases. Some of the diagnostic process will involve ruling out other possible causes of your child’s symptoms. Your doctor may recommend genetic testing as part of the diagnostic process.

How are periodic fever syndromes treated?

There is no cure for many of the periodic fever syndromes, but some of the conditions can be managed successfully with medications that treat or prevent the symptoms. Your child’s rheumatologist will review the specific treatment for your child’s condition. Non-steroidal anti-inflammatory medications (NSAIDs) may also be prescribed to help with inflammation.