What is Periodic Fever Syndrome?

Periodic fever syndrome is the term for a number of conditions characterized by recurrent episodes of unexplained fever. Sometimes these fevers are accompanied by other symptoms, but not always. Periodic fever syndrome usually appears in childhood and may resolve before adulthood, but not always.

These conditions are rarely fatal, but can lead to lifelong challenges and treatment. Read on to learn more about the different types of periodic fever syndromes and how to manage them.

Types of Periodic Fever Syndrome

Periodic fever syndrome is a general term for a series of fever symptoms that are not caused by bacterial or viral diseases. Most of these diseases are hereditary, passed down the family as genetic mutations.

The most common periodic fever syndromes are listed below.

  • Familial Mediterranean Fever (FMF)
  • Hyperimmunoglobulin D Syndrome (HIDS)
  • Muckle-Wells syndrome and familial cold autoinflammatory syndrome
  • Neonatal Multisystem Inflammatory Disease (NOMID)
  • Periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome (PFAPA)
  • Tumor necrosis receptor-associated periodic syndrome (TRAPS)

Periodic Fever Syndrome Symptoms

While the most common symptom in this cluster is recurrent fever without an infectious cause, there are also a range of symptoms that accompany fever, depending on what you may have:

  • FMF: This is the most common periodic fever syndrome that, in addition to fever, includes abdominal pain, arthralgia, and chest pain.
  • TRAPS: Formerly known as familial hibernation fever, in addition to episodic fever, TRAPS can cause abdominal pain, diarrhea, muscle pain, swelling around the eyes, a painful rash, and body aches that move around the body.
  • HIDS: Also known as mevalonate kinase-associated periodic fever syndrome, in which the fever becomes quite high. You may also experience a rash, swollen lymph nodes, headache, loss of appetite, abdominal pain, vomiting, and diarrhea.
  • NOMID: This condition is usually accompanied by fever and rash, but there are many other more serious symptoms. People with NOMID may also have one or more of the following: chronic meningitis, vision problems, hearing loss, other neurological problems, bulging eyes, vomiting, delayed closure of the soft spot (anterior fontanelle) in infancy, Headache, joint pain or swelling, bone or joint deformities, hand deformities, and clubbing of fingers and/or toes.
  • Muckle-Wells Syndrome and Familial Cold Autoinflammatory Syndrome: This condition presents with a fever and sometimes a rash or deafness.
  • PFAPA: In addition to periodic fever, people with PFAPA may experience sore throat, mouth sores, swollen lymph nodes in the neck, headache, vomiting, diarrhea, abdominal pain, and joint pain.

When is a fever too high?

reason

While each periodic fever syndrome is unique, they are usually inherited. In most cases, these diseases run in families and are caused by genetic mutations and are considered autoinflammatory diseases.

Autoinflammatory diseases represent a malfunction within the immune system. People with these diseases have genetic mutations that not only attack foreign threats such as viruses or bacteria, but also cause their immune cells to attack their own bodies. This can cause many symptoms, including fever.

The symptoms that appear depend on the body system your immune cells are fighting. In some cases, genetic abnormalities cause limited symptoms. In other cases, inflammation is more prevalent, causing damage to other organs.

diagnosis

The diagnosis of periodic fever syndrome usually occurs after months or years of unexplained fever without evidence of viral or bacterial infection. These disorders usually appear in infancy or childhood, but can also persist into adulthood.

READ ALSO:  Overview of Reflex Sympathetic Dystrophy (RSD)

With the exception of the more common FMF, most of these conditions are so rare that most doctors don’t see them.

Usually, the diagnosis will rely on family history and the presence of certain symptoms.

Symptoms and tests for diagnosis

A formal diagnosis may be accompanied by the following symptoms or tests:

  • Recurrent febrile pattern without infection observed
  • No other problems, such as cancer, infection, or other illnesses
  • Genetic testing for known genetic mutations
  • family history
  • Ethnicity – FMF prevalence is higher in people of Mediterranean and Middle Eastern ancestry
  • Elevated inflammatory markers such as C-reactive protein

treat

Again, the exact treatment will depend on the specific disease, but these diseases can usually be managed with drugs that reduce inflammation or suppress the immune system. While there are treatments available to manage the symptoms of these illnesses — some may subside with age — most cyclic febrile illnesses are incurable.

There has also been some progress in the use of biologics such as Enbrel (etanercept), Ilaris (canakinumab) and Kineret (anakinra), which block certain immune functions to treat certain periodic fever syndromes.

These drugs reprogram immune cells by targeting and disarming a mutated cell type, or by blocking receptors that respond to those cell types. Biologics are frequently used to manage autoimmune and autoinflammatory diseases.

The following specific treatments may help some conditions.

  • FMF: Colchicine, Iris
  • HIDS: NSAIDs, steroids
  • Muckle-Wells Syndrome and Familial Cold Autoinflammatory Syndrome: Kineret
  • NOMID: NSAIDs, steroids, methotrexate, Kineret
  • PFAPA: Steroids, Cimetidine
  • TRAPS: Anti-inflammatory agents like steroids, Enbrel

Safe Ways to Treat Fever

prognosis

While recurrent fevers can cause noticeable symptoms, they are rarely fatal and can usually be treated with medication. In both PFAPA and HIDS, symptoms usually subside throughout childhood, especially after age 10.

Mild symptoms may persist into adulthood or lead to other conditions such as arthritis. Mild symptoms may persist into adulthood or lead to other conditions such as arthritis. FMF is a lifelong disease that can be controlled with continued treatment with colchicine. Treatment is ongoing.

On the other hand, NOMID often results in severe physical deformities and/or nerve damage. Many of these disorders, especially Muckle-Wells, are also associated with a condition called amyloidosis.

Amyloidosis occurs when abnormal proteins build up and cause damage to your organs, especially your kidneys.The condition can even lead to kidney failure.

While these conditions cause a range of symptoms from mild to severe, periodic fever syndrome is not fatal in most cases.

VigorTip words

Periodic fever syndrome is a series of illnesses with recurrent fevers not caused by infection. These diseases are largely rare and not contagious. Most of these conditions are passed down the family as genetic mutations. Symptoms may persist for life, but anti-inflammatory drugs and new biological therapies can help control them.

What to do if your child has a fever