Management of PFAPA Syndrome

Diagnostic Criteria and Initial Evaluation

The American Academy of Otolaryngology-Head and Neck Surgery recommends documenting accurately every episode in the medical record, including temperature, symptoms, duration, and impact on quality of life 1

The American Academy of Otolaryngology-Head and Neck Surgery suggests that tonsillectomia may be considered as a definitive treatment for patients with persistent symptoms, significant impact on quality of life, history of multiple peritonsillar abscesses, or patient/family preference for a definitive solution 1

Clinicians should not confuse PFAPA syndrome with recurrent streptococcal pharyngitis, as patients with PFAPA may be chronic carriers of streptococcus and experience repeated viral infections 2, 3
The American Academy of Pediatrics recommends avoiding the use of aspirin in children to control fever due to the risk of Reye's syndrome 4, 5
Accurate documentation of each episode, including clinical characteristics, test results, and days of school absence, is crucial for monitoring the frequency and severity of episodes 1

Regular clinical visits are necessary to monitor the frequency and severity of episodes, and to assess the need for therapeutic escalation towards tonsillectomy 1

The American Academy of Otolaryngology-Head and Neck Surgery recommends tonsillectomy as a definitive treatment option for patients with PFAPA syndrome, particularly when symptoms significantly impact quality of life 6, 7
Throat culture to rule out Group A Streptococcal infection is recommended as part of the diagnostic workup for PFAPA syndrome, according to the Infectious Diseases Society of America 8
Anti-streptococcal antibody titers are not recommended for diagnosis as they reflect past rather than current events, as stated by the Infectious Diseases Society of America 8
Diagnostic testing of asymptomatic household contacts is not routinely recommended, according to the Infectious Diseases Society of America 8
Tonsillectomy should be considered for patients with persistent symptoms despite medical management, as recommended by the American Academy of Otolaryngology-Head and Neck Surgery 7
The American Academy of Otolaryngology-Head and Neck Surgery suggests considering tonsillectomy for patients with a significant impact on quality of life 6
No need for routine post-treatment throat cultures, as stated by the Infectious Diseases Society of America 8

The American Academy of Otolaryngology-Head and Neck Surgery recommends tonsillectomy as the most effective definitive treatment for PFAPA syndrome, achieving complete symptom resolution in the vast majority of patients, especially those with significant quality of life impact, persistent symptoms, or history of peritonsillar abscess 9
Tonsillectomy is favored even when patients don't meet standard frequency criteria for recurrent throat infections, according to the American Academy of Otolaryngology-Head and Neck Surgery 9

The American Academy of Otolaryngology-Head and Neck Surgery strongly recommends against prescribing perioperative antibiotics for tonsillectomy, and instead suggests administering a single intraoperative dose of IV dexamethasone and using ibuprofen, acetaminophen, or both for postoperative pain control 9
A history of more than one peritonsillar abscess is a specific indication for tonsillectomy, as recommended by the American Academy of Otolaryngology-Head and Neck Surgery 9