| Essential features |
|---|
| Recurrent fever: ≥ 3 episodes in 6 months; duration of 3–5 days |
| Exclusion of PFAPA, monogenic HRF and cyclic neutropenia |
| Asymptomatic interval between fever episodes |
| Normal growth and development |
| Additional features |
|---|
| Sore mouth, pharyngitis, or adenitis (i.e., PFAPA triad) |
| Respiratory symptoms ± past history of allergic diseases |
| Abdominal pain, nausea, or vomiting |
| Arthralgia or fatigue |
| Eye manifestations: Conjunctivitis or eyelid swelling |
| Response to treatment (e.g., steroids, colchicine, or anakinra) |