From the Research
Age Range for PFAPA Syndrome
The age range for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome is typically characterized by:
- Onset usually before the age of 5 years 1, 2, 3, 4
- Most cases resolve spontaneously before age 10 1
- However, there are reports of PFAPA syndrome in adults, with a study describing 15 adult patients diagnosed with the syndrome, with episodes of fever occurring at 4.6 +/- 1.3 week intervals, beginning at the age of 20.9 +/- 7.5 1
- A review of the literature suggests that typical PFAPA syndrome is not associated with significant long-term sequelae and has a good response to steroids, with the age of onset typically less than 5 years 2
- Another study found that the median age at onset was 1.58 years, and the median age at diagnosis was 2.97 years, with a higher frequency of febrile attacks in children with an age at diagnosis under 1 year 5
Key Characteristics
Some key characteristics of PFAPA syndrome include:
- Periodic fever, aphthous stomatitis, pharyngitis, and adenitis typically before age 5 3
- Elevated C-reactive protein in the absence of elevated procalcitonin, vitamin D, CD64, mean corpuscular volume, and other nonspecific inflammatory mediators in the absence of an infectious explanation for fever 3
- Effective treatment includes steroids, tonsillectomy/adenoidectomy, and cimetidine 2
- Tonsillectomy remains the only permanent treatment modality 3