General Information About PFAPA Syndrome
Disease Details and Frequently Asked Questions
Named after its English acronym (Periodic Fever, Aphthous stomatitis, Pharyngitis, and Adenitis), it is the most common autoinflammatory syndrome seen in children, presenting with regular episodes of fever and throat/mouth inflammation.
It is almost as predictable as a clock. While the child is completely healthy, every 3-4 weeks (usually every 21-28 days), they suddenly experience high fever episodes spiking to 39-40 degrees Celsius, lasting for about 4-5 days.
In a normal throat infection, administering antibiotics lowers the fever and cures the illness. Because PFAPA attacks are not bacterial in origin, they absolutely do not respond to antibiotics, and even fever reducers do not provide any significant benefit.
No. Unlike FMF (Familial Mediterranean Fever), it does not cause severe organ damage or growth retardation. Outside of the attacks, the child is completely healthy, and the disease usually disappears on its own by the time the child reaches their teenage years.
There is no specific blood test. Diagnosis is made by the regularity of the attacks, negative throat cultures (absence of bacteria), and the physician's clinical observation of classic symptoms (mouth ulcers, swollen neck glands).
When an attack begins, a single low dose of oral cortisone (steroid) miraculously reduces the fever to zero within a few hours; this acts as both a treatment and a confirming test for the PFAPA diagnosis. In children who experience very frequent attacks and have a reduced quality of life, surgical removal of the tonsils (Tonsillectomy) largely eliminates the disease completely.
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