Table 1 CTPs inclusion/exclusion criteria for patients with PFAPA

Patient Characteristics (All criteria must be fulfilled)

• 3–7 days of fever (minimum fever of 102.2 °F (39 °C) for 3 days per episode)

• ≥ 6 episodes occurring with regularity

• Definition of Regularity:

 • If fever cycle is 2–4 weeks, 1 week of variability would be allowed. If fever cycle is 5–8 weeks, 2 weeks of variability will be allowed.

 • Should have associated pharyngitis. In the absence of pharyngitis, patient must have both aphthous stomatitis and cervical adenopathy.

• Normal growth and development

• Steroid Responsive (if corticosteroids are administered with an episode)

 - Definition of steroid responsiveness - patient will be considered steroid responsive if fever resolves within 24 h after a maximum steroid dose of 2 mg/kg (max 60 mg) given in a single dose or divided over 2 doses.

Patients should NOT have

• Other known autoimmune/autoinflammatory disorder

• Immunodeficiency (e.g cyclic neutropenia)

• Malignancy

• Infection