Table of contents
The following summarized guidelines for the evaluation and management of PFAPA syndrome are prepared by our editorial team based on guidelines from the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF 2019), the PFAPA Syndrome Working Group (PFAPA-WG 2016), and the American Academy of Allergy, Asthma & Immunology (AAAAI/ACAAI 2015).
1.Screening and diagnosis
Diagnosis: as per PFAPA-WG 2016 guidelines, suspect PFAPA syndrome in pediatric patients with recurrent and periodic febrile episodes of unknown origin, or with recurrent tonsillitis, interspersed with asymptomatic periods, especially in patients with a good general condition and with preservation of weight and height development.
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Corticosteroids: as per PFAPA-WG 2016 guidelines, initiate prednisone (1-2 mg/kg body weight) at the onset of fever in patients with PFAPA syndrome.
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Indications for tonsillectomy: as per AAO-HNSF 2019 guidelines, assess pediatric patients with recurrent throat infections not meeting the criteria for tonsillectomy for PFAPA syndrome that may nonetheless favor tonsillectomy.