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PFAPA syndrome

Key sources
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).
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Guidelines

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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2.Medical management

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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3.Surgical interventions

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.
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