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 |