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. 2022 Jul 18;11(14):2231. doi: 10.3390/cells11142231

Table 8.

Definition of the periodic fever/aphthous stomatitis/pharyngitis/cervical adenopathy (PFAPA) syndrome in children, in adults and according to the Eurofever/PRINTO classification criteria.

PFAPA syndrome in children Periodically recurring high fevers (with “clockwork” periodism at intervals of 4–6 weeks)
+
Onset before 5 years
+
Child’s complete wellness between attacks (with normal growth and no sequelae)		 At least 1 among:
  • (a)

    aphthous stomatitis

  • (b)

    pharyngitis

  • (c)

    cervical lymphadenitis

Absence of respiratory infection-related symptoms		 To rule out:
  • -

    Cyclic neutropenia

  • -

    Recurrent upper respiratory infections

  • -

    Monogenic hereditary sautoinflammatory disorders
PFAPA syndrome in adults
(at least 16-year-old)		 Recurrent fevers
+
Increased inflammatory parameters during febrile attacks
+
Symptom-free intervals		 At least 1 between:
  • -

    pharyngitis

  • -

    cervical lymphadenitis

 To rule out:
  • -

    Infections

  • -

    Autoimmune disorders

  • -

    Monogenic hereditary autoinflammatory disorders
Eurofever/PRINTO classification criteria for PFAPA syndrome At least 7 out of the following 8 signs (either positive [from a to d] or negative [from e to h]):
  • (a)

    pharyngotonsillitis

  • (b)

    febrile flares lasting 3–6 days

  • (c)

    cervical lymphadenitis

  • (d)

    periodic recurrence of flares
  • (e)

    absence of diarrhea

  • (f)

    absence of chest pain

  • (g)

    absence of skin rash

  • (h)

    absence of arthritis