Table 1.

Comparison of three classification criteria of PFAPA syndrome

classification criteria	Modified Marshall’s	Vanoni et al	Takeuchi et al
Fever	Regular recurrent fevers	Periodic Fever for at least 6 months: a. fever ≥ 38.5 °C b. Regular recurrent fevers ≥ 5 episodes, interval between fevers ≥ 2 months, Asymptomatic between episodes	Fever ≥ 38.0 °C that Recurs ≥ 4 times Asymptomatic between episodes
Duration of fever	-	2–7 days	< 8 day
Symptoms	Constitutional symptoms, absence of infection, ≥ 1 of the following signs: 1.Apthous stomatitis 2.Cervical lymphadenitis 3.Pharyngitis	Pharyngitis, cervical adenitis, Aphthous stomatitis, ≥ 1 in every episodes and ≥ 2 from 3 in the majority of episodes	Tonsillitis or pharyngitis Accompanying symptoms with ≥ 1 of the following signs 1.Aphthous stomatitis 2.cervical lymphadenitis 3.sore throat 4.vomiting 5.severe headache 6.No cough
Exclusion	Cyclic neutropenia	Other causes of recurrent fever	-
Growth and development	Normal Growth and development	Normal Growth
Family history	-	-	A history of recurrent fever
laboratory Tests			Inflammatory findings (CRP & SAA) during febrile episodes Elevations of IgD
corticosteroids during febrile episode	-	-	Significant effect

CRP C‐reactive protein, SAA Serum amyloid, IgD Immunoglobulin D