What is periodic fever syndrome?
PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) is a childhood syndrome that affects both boys and girls. It causes repeated episodes of fever, mouth sores, sore throat, and swollen lymph nodes. PFAPA usually starts in early childhood between ages 2 and 5.
How do you test for PFAPA?
How is PFAPA syndrome diagnosed?
- Blood tests, such as white blood counts. These look for signs of infection.
- Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). These blood tests check for general inflammation.
- Strep culture. This checks for strep throat.
- Imaging tests.
- Genetic tests.
How do I know if my child has periodic fever syndrome?
Children with periodic fever syndrome have recurrent fevers that usually last the same amount of time and are accompanied by the same or similar symptoms with each episode of fever. Those symptoms include swelling, joint pain, sores in the mouth or a rash.
What triggers PFAPA?
PFAPA is an autoinflammatory, not autoimmune, disease and does not have a known trigger. Due to inappropriate activation and regulation of antigen-independent inflammation (innate immunity).
Can vaccines trigger PFAPA?
Vaccines triggered the disease in 70% of the MKD, while PFAPA, TRAPS and UND had a rate of reactions of 20%. This was also found in 12.34% of CAPS, whereas FMF and inflammatory bone disorders had a rate of 6% and 3%, respectively.
How many periodic fever syndromes are there?
Hereditary periodic fevers were once grouped based on clinical findings and Mendelian patterns of inheritance into nine distinct groups. Syndromes with an autosomal-dominant pattern are as follows: Tumor necrosis factor receptor–associated periodic syndrome (TRAPS) Familial cold autoinflammatory syndrome (FCAS)
What kind of doctor treats PFAPA?
Usually, a rheumatology clinic will have a team of doctors and nurses who have a lot of experience treating children with PFAPA.
Is PFAPA curable?
PFAPA usually resolves spontaneously during the second decade of life. However, the disorder may continue into adulthood, although episodes usually become less frequent. Steroids given at the start of an episode usually end it, but the result may be more frequent episodes.
Is FMF an autoimmune disease?
As opposed to an autoimmune disease in which the immune system attacks its own cells, FMF is an autoinflammatory disease in which the innate immune system (the body’s first-line defense) simply doesn’t work as it is should.
Can PFAPA be cured?
There is no medication that can cure PFAPA. Steroids like prednisone given at the start of an attack can shorten the length of the attack quite a bit. Sometime two doses, 24 hours apart, are needed. However, the attacks may happen a little more often if steroids have been used.
Is PFAPA immunocompromised?
Is PFAPA an immunodeficiency? In the cases of PFAPA patient’s immunity is not decreased. As the immune system does not work properly – it is excessively responsive – we consider it as one of the immune system disorders.
Is PFAPA a rare disease?
The exact prevalence of PFAPA is not known, but the disease appears to be more common than originally thought, and PFAPA may be the most frequent recurrent autoinflammatory fever syndrome in children. Despite many patients having persistent disease for years, PFAPA is generally thought to be relatively benign.
How do you treat periodic fever?
There is no specific treatment to cure PFAPA. The aim of treatment is to control symptoms during the episodes of fever, to shorten the duration of episodes, and in some children to prevent attacks from occurring. In most children, the disease will resolve by itself without treatment, usually after the age of 10 years.
Is FMF fatal?
With early and regular treatment, individuals with FMF can live a normal lifespan and may even be free of symptoms. The disease has the potential to be life-threatening if the patient develops kidney failure (which may result when a person is untreated or does not respond to treatment).
Can PFAPA cause seizures?
Two PFAPA episodes were associated with aseptic encephalitis and seizures. Recurrent acute aseptic encephalitis or seizures have never been reported during the febrile episodes of PFAPA. This possible association is discussed within the context of the etiology of PFAPA.
Is PFAPA auto inflammatory?
The polygenic autoinflammatory condition of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is the most common autoinflammatory disorder among children in many parts of the world.
Does PFAPA run in families?
Although the majority of case subjects of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome are considered sporadic, familial case subjects are reported.
Is PFAPA genetic?
PFAPA syndrome is the most common autoinflammatory syndrome in children from Western countries. In spite of its strong familial clustering, its genetic basis and inheritance pattern are still unknown.