PFAPA syndrome in children: A meta-analysis on surgical versus medical treatment

Stamatios Peridis1, Gemma Pilgrim, Emmanouel Koudoumnakis

  • 1Department of Otolaryngology Head and Neck Surgery, Guy's and St Thomas' NHS Foundation Trust, London, United Kingdom. peridis@gmail.com

Insights

For children with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome, tonsillectomy offers the most effective long-term symptom resolution. Corticosteroids provide temporary relief but do not prevent future fever cycles.

Area of Science:

  • Pediatric immunology
  • Otolaryngology
  • Clinical effectiveness research

Background:

  • Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome is a common autoinflammatory disorder in children.
  • Treatment options for PFAPA syndrome are varied, necessitating a comparison of their efficacy.

Purpose of the Study:

  • To compare the effectiveness of medical and surgical therapies for managing PFAPA syndrome in pediatric patients.

Main Methods:

  • A comprehensive literature search was conducted across major databases (Medline, Embase, Ovid, Cochrane) for studies published between 1987 and 2010.
  • Random-effect meta-analytical techniques were employed to analyze outcome measures comparing surgical versus medical treatments for PFAPA syndrome.

Main Results:

  • Antibiotics and cimetidine were found to be ineffective for PFAPA syndrome.
  • Corticosteroids demonstrated effectiveness in symptom resolution, while tonsillectomy (+/- adenoidectomy) showed significant efficacy (P<0.00001).
  • Meta-analysis confirmed surgery as significantly more effective than antibiotics or cimetidine; no significant difference was found between steroids and surgery (P=0.83).

Conclusions:

  • Corticosteroids are the most effective non-surgical treatment for PFAPA syndrome, but do not prevent recurrence.
  • Tonsillectomy (+/- adenoidectomy) is identified as the most effective intervention for achieving long-term resolution of PFAPA syndrome symptoms.
Abstract