What is PFAPA Syndrome?
PFAPA is a disease that causes regular fever attacks. Attacks are often accompanied by swollen neck lymph nodes, sore throat, and/or sores in the mouth. Fever attacks begin suddenly and last three to seven days. Fever occurs routinely, usually every three to six weeks. Families usually know exactly when an attack will begin. Some children have other symptoms such as joint pain, abdominal pain, headache, vomiting, or diarrhea. During attacks, children look quite sick, but in the periods between attack fevers, they look completely healthy.
What is the Cause of PFAPA Syndrome?
It is not yet known what causes this disorder. For reasons not yet understood, the body's inflammatory system becomes active, leading to PFAPA symptoms. PFAPA is not inherited. A gene defect in PFAPA has not yet been found, but sometimes more than one family member can have the disease. PFAPA is not caused by any infection that we know of and is not an infectious disease. It is clear that the inflammatory process is active during attacks, but the reason for this is unknown.
What Course Does PFAPA Syndrome Follow?
The disease may last for several years, but it usually resolves spontaneously in the second decade of life. However, in approximately 15% of patients, fever attacks continue into adulthood. Over time, the time between attacks becomes longer. Children with PFAPA continue to grow and develop normally.
How is PFAPA Syndrome Diagnosed?
There are no laboratory tests specific to diagnosing PFAPA. The disease is diagnosed based on symptoms and physical examination. White blood cell counts and markers of inflammation, including sedimentation rate and C-reactive protein, all of which can be measured with a blood test, are elevated during fever attacks. It is important to rule out all other diseases (especially beta streptococcal infection) that may present with similar symptoms before confirming the diagnosis. The dramatic response to treatment with steroids also helps diagnose PFAPA. In cases where the classic disease attack pattern is not present, especially if symptoms begin in the first year of life and there is inflammation between attacks If markers are also increased, other recurrent It is necessary to exclude the causes of fever.
How is PFAPA Syndrome Treated?
The aim of the treatment will be to control the symptoms during fever attacks, shorten the duration of the attacks and prevent the attacks from occurring. Fever usually does not respond well to medications such as acetaminophen or ibuprofen. A single dose of steroids given when symptoms first begin can dramatically shorten and often end a fever attack. However, with this treatment, the time between episodes may also be shortened and the next episode may occur earlier than expected. Antibiotics are not effective. Typical attacks of PFAPA should not be treated with antibiotics. However, children with PFAPA can also get common childhood infections. Consult your child's doctor to determine whether the fever attack is PFAPA.
Report the diagnosis to your child's school. Children with PFAPA are not contagious and should not be excluded from school.
It is always best to seek advice from a Rheumatologist for more information and before making any decisions about treatment and follow-up of the patient.
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