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Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome

Nora S Ali1, Julio C Sartori-Valinotti2, Alison J Bruce3

  • 1Mayo Medical School, Mayo Clinic, Rochester, MN.

Clinics in Dermatology
|June 26, 2016
PubMed

Insights

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a common childhood disorder. Recent literature highlights its immune and genetic links, with tonsillectomy as the only permanent treatment.

Area of Science:

  • Pediatric Rheumatology
  • Immunology
  • Genetics

Background:

  • Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most frequent periodic disorder in children.
  • Symptoms typically manifest before age five, including fever, aphthous ulcers, pharyngitis, and adenitis.

Purpose of the Study:

  • To review recent literature on PFAPA syndrome.
  • To summarize key findings regarding its pathogenesis, evaluation, and treatment.

Main Methods:

  • Literature review of recent studies on PFAPA syndrome.
  • Analysis of proposed pathogenetic mechanisms, diagnostic biomarkers, and therapeutic interventions.

Main Results:

  • Pathogenesis theories involve innate immune dysfunction and T-cell dysregulation, with potential genetic links, including MEFV gene variants influencing severity.
  • Diagnostic criteria incorporate clinical signs and biomarkers like elevated C-reactive protein (without elevated procalcitonin), vitamin D, CD64, and mean corpuscular volume, excluding infections.
  • Treatment options include corticosteroids, IL-1 blockers (anakinra, rilonacept, canakinumab), and tonsillectomy.

Conclusions:

  • PFAPA syndrome pathogenesis is multifactorial, involving immune and genetic components.
  • Accurate diagnosis relies on clinical presentation and specific biomarkers.
  • Tonsillectomy is the sole permanent treatment, while other options provide symptomatic relief.

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