What triggers periodic fever syndrome?

periodic fever syndrome - Sriramakrishnahospital

The phrase periodic fever syndrome refers to a group of disorders characterized by recurrent, unexplained fever. However, high fevers are not usually accompanied by additional symptoms. Syndromes of periodic fever typically manifest in childhood and may resolve before the onset of adulthood, however, this is not always the case.

These disorders are infrequently fatal, although they can cause lifetime difficulties and therapies. Learn more about the many types of recurrent fever syndromes and how to treat them by reading on.

Types of Periodic fever syndrome:

Periodic fever syndrome is an umbrella name for various illnesses characterized by a fever not caused by a bacterial or viral infection. The majority of these illnesses are genetic mutations that are passed down through families.

The following are the most prevalent periodic fever syndromes.

  • Familial Mediterranean fever (FMF)
  • Hyper immunoglobulin D syndrome (HIDS)
  • Muckle-Wells syndrome and familial cold autoinflammatory syndrome
  • Neonatal onset multisystem inflammatory disease (NOMID)
  • Periodic fever, aphthous-stomatitis, pharyngitis, adenitis syndrome (PFAPA)
  • Tumor-necrosis-receptor-associated rare syndrome (TRAPS)

Causes of periodic fever syndrome:

Although each of the periodic fever syndromes is distinct, they are frequently inherited. In most cases, these conditions are inherited and caused by a gene mutation; they are categorized as autoinflammatory diseases.

Autoinflammatory disorders indicate a type of immune system dysfunction. In persons with these disorders, gene abnormalities enable their immune cells to target their bodies in addition to other threats such as viruses and bacteria. This may cause a variety of symptoms, including fevers. Periodic fever syndrome in babies is quite common when compared.

Signs that indicate periodic fever syndrome:

While recurring fever without an infectious cause is the most prevalent symptom of this class of illnesses, there are a variety of other symptoms associated with fevers, depending on the condition:


In addition to fevers, this is the most prevalent of the periodic fever syndromes and is characterized by abdominal pain, joint pain, and chest pain.


In addition to episodic fevers, TRAPS, formerly known as familial Hibernian fever, can produce abdominal pain, diarrhea, muscle soreness, swelling around the eyes, painful skin rashes, and widespread body pain.


This disorder, also known as mevalonate kinase-associated periodic fever syndrome, can cause extremely high fevers. You may also develop skin rashes, lymph node enlargement, headache, appetite loss, abdominal pain, vomiting, and diarrhea.


People with NOMID may also have persistent meningitis, vision issues, hearing loss, other neurologic abnormalities, bulging eyes, vomiting, delayed closure of the soft spot (anterior fontanel) in infancy, headaches, and joint discomfort, or edema.


PFAPA patients may also experience a sore throat, mouth ulcers, enlarged lymph nodes in the neck, headache, vomiting, diarrhea, abdominal pain, and joint pain in addition to periodic fevers.

Recommended diagnostic procedures to confirm periodic fever syndrome:

Physical exam:

Your child’s rheumatologist will do a comprehensive physical examination to look for signs of the disease.

Review of family medical history:

Certain varieties of recurrent fever syndrome (including FMF) are typically limited to individuals with a particular ancestry, such as those of Mediterranean origin. This is why discussing your family’s medical history with your child’s best rheumatologist doctor can aid in periodic fever syndrome diagnosis.

Blood test:

Your child’s best rheumatologist doctor may examine his or her blood for signs of inflammation, such as elevated numbers of white blood cells.

Genetic test:

Genetic testing may be utilized as a supplemental diagnostic technique. In conjunction with other tests, it can help detect if your child contains the gene responsible for certain diseases.

Treatment options that can involve in treating periodic fever syndrome:

Again, the precise treatment for periodic fever syndrome that is utilized will depend on the disease that is being treated; but, in general, drugs that lower inflammation or suppress the immune system may be used to control these conditions. 

There are therapies available to control the symptoms of these conditions, and some of them may resolve on their own with age; but, the majority of periodic fever syndromes cannot be cured.

At times biologics treatment may also be recommended by the best rheumatologist doctor,


In the treatment of certain recurrent fever syndromes, biologic therapy is recommended. Protein medicines are known as biologics, and they can be administered in a variety of ways, such as an injection just under the skin (similar to how insulin is administered) or as an infusion through an intravenous (IV) catheter. 

In most cases, elevated levels of inflammatory proteins are related to the presence of periodic fever syndromes. Biologics use a more direct approach than conventional drugs to address these proteins.

What triggers periodic fever syndrome?

There is a gene called MEFV that has a protein called pyrin. When this gene is defective, the regulation of inflammation cannot be carried out adequately, resulting in fever and other symptoms. Infection, trauma, menstruation (periods), or psychological stress might cause an episode.

Periodic fever syndromes are a group of illnesses characterized by noninfectious recurring fevers. These conditions are uncommon and not communicable. 

The majority of these illnesses are inherited as genetic mutations. Symptoms may last for a lifetime, although anti-inflammatory medications and novel biologic therapies can help manage them. Reach out to the best rheumatologist doctor in Coimbatore to undergo the treatment of the condition.

periodic fever syndrome - Sriramakrishnahospital

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