Table III.

Proposed diagnostic criteria for adult-onset PFAPA according to Cantarini [14]

1. Age ≥ 16

2. Recurrent fever accompanied by erythematous pharyngitis and/or cervical lymphadenitis

3. Increased inflammatory markers during attacks

4. Intervals between disease flare without any symptoms

Exclusions:

infections, autoimmune and neoplastic diseases as well as monogenic autoinflammatory diseases and febrile polygenic autoinflammatory diseases

throat swab performed during fever has to be negative and antibiotic therapy considered ineffective