Guideline 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). 1 2 3 Calculator Calculator FeverPAIN score for streptococc Modified Centor score 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. E Show 2 more https://web.pathway.md/diseases/rec5QUgZZIY6FrQnm 1/3 6/29/23, 10:50 PM PFAPA syndrome Pathway As per AAAAI 2015 guidelines, suspect PFAPA syndrome in young pediatric patients presenting with characteristic clinical features: a prodrome of cardinal features, including fatigue, chills, and oral ulcers on the lips and buccal mucosa, followed by cervical lymphadenopathy, pharyngitis, and high fever. B 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. E As per AAAAI 2015 guidelines, initiate oral corticosteroids for the management of acute episodes of PFAPA syndrome. B Interleukin-1 inhibitors: insufficient evidence to support the use of IL-1 beta inhibitors for the management of patients with PFAPA syndrome. I 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. B As per PFAPA-WG 2016 guidelines, consider offering tonsillectomy (with or without adenoidectomy) for the resolution of symptoms in patients with PFAPA syndrome on an individual basis (for example, when affecting the quality of life or in case of poor response to medical treatment). E Clinical findings Patient demographics Symptoms Children Abdominal pain Arthralgia Head and neck exam Chills Cough Aphtous ulcers Diarrhea Oral ulcers Fatigue Pharyngeal erythema Fever Pharyngeal exudate Flu-like symptoms Pharyngitis Headache Lab findings Irritability Malaise ESR Skin rash serum CRP Sore throat Vomiting https://web.pathway.md/diseases/rec5QUgZZIY6FrQnm 2/3 6/29/23, 10:50 PM PFAPA syndrome Pathway Lymphatic exam Cervical lymphadenopathy Hematological findings Monocytosis WBC count blood neutrophil count blood lymphocyte count References 1. Mitchell RB, Archer SM, Ishman SL et al. Clinical Practice Guideline: Tonsillectomy in Children Update). Otolaryngol Head Neck Surg. 2019 Feb;160 1_suppl):S1 S42. Open 2. Maria Teresa R A Terreri, Wanderley Marques Bernardo, Claudio Arnaldo Len et al. Guidelines for the management and treatment of periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome. Rev Bras Reumatol Engl Ed. 2016 Jan-Feb;56 1 52 7. Open 3. Francisco A Bonilla, David A Khan, Zuhair K Ballas et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015 Nov;136 5 1186 205.e1 78. Open https://web.pathway.md/diseases/rec5QUgZZIY6FrQnm 3/3