From the Guidelines
PFAPA syndrome typically affects children between the ages of 2 and 5 years old, but it can occur in children as young as 1 year and as old as 12 years. The condition usually resolves spontaneously by adolescence, with most cases resolving by age 10-12 1. While PFAPA is primarily a pediatric condition, there have been rare reports of adult-onset cases. However, these are considered atypical and should prompt investigation for other periodic fever syndromes.
Key points to remember:
- Most common onset: 2-5 years old
- Possible range: 1-12 years old
- Typical resolution: By adolescence (10-12 years) The age range for PFAPA is related to the development of the immune system. The syndrome is thought to be caused by dysregulation of the innate immune response, which is still maturing in young children. As the immune system develops and becomes more regulated with age, the symptoms often improve or resolve completely. For diagnosis and management, it's crucial to consider the typical age range, as PFAPA can be mistaken for other conditions. Regular follow-up is important, as treatment strategies may need to be adjusted as the child grows and the condition potentially resolves. According to the most recent clinical practice guideline: tonsillectomy in children (update) 1, PFAPA is considered a modifying factor that may favor tonsillectomy in children with recurrent throat infection.
From the Research
Age Range for PFAPA Syndrome
The age range for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome is typically characterized as follows:
- The age of onset is usually less than 5 years, with a median age of 24 months 2
- Most cases are resolved by the age of 10 years 3
- The syndrome can occur in adults, although it is rare 3
- Some studies suggest that the syndrome can appear before the age of 5 and has a limited duration of 4-8 years 4
Key Characteristics of PFAPA Syndrome
Some key characteristics of PFAPA syndrome include:
- Recurrent episodes of fever associated with pharyngitis, stomatitis, and cervical adenitis 2, 5, 3
- Episodes can last for days or even weeks, with asymptomatic periods in between 4
- The syndrome is benign and can be diagnosed by detailed history-taking and physical findings during repeated febrile episodes 5
Diagnosis and Treatment
Diagnosis of PFAPA syndrome is made clinically, as there are no specific biomarkers available 3. Treatment options include: