What are periodic fever syndromes (now known as recurrent fever syndromes)?
Recurrent fever syndromes, formerly known as periodic fever syndromes, are a group of disorders that cause recurrent fevers that don’t have an infectious (virus, bacteria) cause. The syndromes are considered autoinflammatory diseases, which means an abnormality or regulation problem in your child’s natural (innate) immune system causes the condition. Recurrent fever syndrome isn’t an autoimmune disease, in which your child’s acquired (adaptive) immune system malfunctions and mistakenly attacks healthy tissue.
Recurrent fever syndromes typically (but not always) start when your child is a baby or toddler. Your child will experience episodes, or attacks, that cause fevers and other symptoms. Children may not have symptoms between episodes. Most of these syndromes are passed down (hereditary) and result from a genetic change (mutation).
Many of these syndromes have a specific treatment, often based on understanding the issue that the genetic mutation causes. If left untreated, periodic fever syndrome can be dangerous because of injury caused by recurrent or ongoing inflammation.
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Is recurrent fever syndrome common?
There are several different types of recurrent fever syndrome. For some types, the frequency (how often someone has the condition) is unknown. For others, the frequency is increasing because researchers have discovered a variety of mutations that cause the syndromes. Most are very rare, but some are more common than others. For instance, tumor necrosis factor receptor-associated periodic syndrome (TRAPS) affects 1 in 1 million people. But familial Mediterranean fever affects approximately 1 in 200 people of Mediterranean and Middle Eastern descent.
What are some of the different types of recurrent fever syndrome?
Familial Mediterranean fever (FMF)
Familial Mediterranean fever (FMF) is the most common genetically diagnosed recurrent fever syndrome. It causes recurrent fevers and may cause painful inflammation in your child’s abdomen, chest and joints. With FMF, the attacks typically begin in childhood. As your child ages, the frequency and severity of their attacks may become less intense.
Periodic fever, aphthous-stomatitis, pharyngitis, adenitis (PFAPA)
Periodic fever, aphthous-stomatitis, pharyngitis, adenitis (PFAPA) includes periodic fevers with mouth sores (aphthous stomatitis), sore throat (pharyngitis) and swollen lymph nodes (adenitis) in your child’s neck. PFAPA affects children in early childhood, usually starting before 4 years old. Episodes usually decrease in frequency and resolve after age 10.
Tumor necrosis factor receptor-associated periodic syndrome (TRAPS)
Apart from episodes of fever, tumor necrosis factor receptor-associated periodic syndrome (TRAPS) may cause abdominal pain, diarrhea, spreading skin rashes, muscle pain and swelling around your child’s eyes. TRAPS may first appear during early to late childhood or into adulthood. The former name of this syndrome was familial Hibernian fever.
Hyperimmunoglobulin D syndrome (HIDS)
Hyperimmunoglobulin D syndrome (HIDS) causes episodic high fevers with skin rashes, swollen neck lymph nodes, abdominal pain, arthritis and/or vomiting and diarrhea. The disease typically starts before 1 year of age. The name of this disease comes from the fact that most babies produce very high amounts of immunoglobulin type D (but this isn’t important diagnostically or clinically). Immunoglobulins are proteins that are part of your child’s immune system.
Neonatal onset multisystem inflammatory disease (NOMID)
Neonatal onset multisystem inflammatory disease (NOMID) causes episodes of fever and skin rash. The symptoms of this condition start at birth or within the first weeks of life. The same gene mutation causes two other diseases (Muckle-Wells syndrome and familial cold autoinflammatory syndrome). These three diseases are called cryopyrin-associated autoinflammatory diseases.
FAQs
Recurrent fever syndromes (periodic fever syndromes) are a group of disorders that cause recurrent fevers. The syndromes are autoinflammatory diseases. Most of these conditions are hereditary and result from a gene mutation.
What are periodic fever syndromes? ›
Abstract. Periodic fever syndromes are autoinflammatory diseases. The majority present in infancy or childhood and are characterised by recurrent episodes of fever and systemic inflammation that occur in the absence of autoantibody production or identifiable infection.
How do you treat recurrent fever syndrome? ›
Periodic fever syndromes include several auto-inflammatory conditions. They often begin in childhood and can cause recurrent severe fevers. To treat periodic fever syndromes, a healthcare professional may prescribe medications, including corticosteroids, NSAIDs, colchicine, and biologics.
What diseases cause recurrent fevers? ›
Understanding Periodic Fever Syndromes
- Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome. PFAPA is probably the most common disorder. ...
- Familial Mediterranean Fever (FMF) ...
- Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS) ...
- Hyperimmunoglobulin D Syndrome (HIDS)
PFAPA (Periodic fever - aphthous stomatitis- pharyngitis - adenopathy) syndrome is an auto inflammatory syndrome characterized by recurrent febrile episodes associated with aphthous stomatitis, pharyngitis and cervical adenitis. ORPHA:42642. Synonym(s): Marshall syndrome with periodic fever.
Can you grow out of periodic fever syndrome? ›
Even without treatment, PFAPA usually stops sometime after age 10. In rare cases, a child may still have symptoms into adulthood, though usually less often.
How long do fevers last with periodic fever syndrome? ›
Periodic fever can be defined as repetitive episodes of fever with no symptoms in between the episodes. These episodes generally last for a few days to weeks up to several months [32,33].
What autoimmune disease causes fever? ›
Rheumatic fever is an autoimmune disease seen in some cases after an infection with Streptococcus bacteria that can affect joints, heart, skin, and brain. This is also called acute rheumatic fever, as fever is one of the main symptoms of the disease.
What cancers cause recurrent fevers? ›
Among cancers, the common culprits for neoplastic or paraneoplastic fever include: Castleman's disease, Hodgkin's and non-Hodgkin's lymphoma, renal cell carcinoma, hepatocellular carcinoma, acute myeloid leukaemia, hairy cell leukaemia, glioblastoma multiforme, blast crisis of chronic myelogenous leukemia, ovarian ...
Is periodic fever syndrome rare? ›
Periodic fever syndrome is a group of disorders in which a child has recurrent episodes of fever over time, usually accompanied by the same symptoms. Each episode of fever usually lasts roughly the same length of time. These disorders are genetic conditions that are very rare.
Recurrent fever syndromes (periodic fever syndromes) are a group of disorders that cause recurrent fevers. The syndromes are autoinflammatory diseases. Most of these conditions are hereditary and result from a gene mutation.
What illness causes a fever to come and go? ›
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.
Why am I getting a fever again and again? ›
Infection is not the only cause of fever. Fever may also result from inflammation, a reaction to a drug, an allergic reaction, autoimmune disorders (when the body produces abnormal antibodies that attack its own tissues), and undetected cancer (especially leukemia, lymphoma, or kidney cancer).
What triggers periodic fever syndrome? ›
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)
What blood test for recurrent fever? ›
The laboratory work-up in children with recurrent fever depends on the history and physical examination. The apparently healthy child needs only a complete blood cell count (CBC) with differential and platelet count, and ESR and C-reactive protein testing. Urine cultures are also useful.
Does Vitamin D help PFAPA? ›
There may be an association between Vitamin D deficiency and a higher frequency of PFAPA episodes. Vitamin D supplementation in children with PFAPA may reduce the frequency of episodes and help manage the condition.
What triggers Pfapa syndrome? ›
Researchers aren't sure what triggers PFAPA. PFAPA doesn't appear to run in families, unlike some other causes of repeated fevers in children. Researchers haven't identified a gene linked to PFAPA. The immune response is different than with a normal infection.
What is autoinflammatory syndrome with periodic fever? ›
Periodic fever syndromes are a heterogeneous collection of conditions that range from monogenic autoinflammatory diseases due to a genetic mutation causing disordered inflammation to benign syndromes that children eventually outgrow.
Why does my fever come and go every few hours? ›
Intermittent fever is a type or pattern of fever in which there is an interval where temperature is elevated for several hours followed by an interval when temperature drops back to normal. This type of fever usually occurs during the course of an infectious disease.
