Table 5. Indications for clinical suspicion of SURF in children with recurrent fever [3335]

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 fatigue1)
 Eye manifestations: Conjunctivitis or eyelid swelling
 Response to treatment (e.g., steroids, colchicine, or anakinra)
Broderick et al.[34] reported that arthralgia and fatigue were less common than gastrointestinal symptoms.
SURF: syndrome of undifferentiated recurrent fever; PFAPA: periodic fever, aphthous stomatitis, pharyngitis, and adenitis; HRF: hereditary recurrent fever.