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Table 1 CTPs inclusion/exclusion criteria for patients with PFAPA

From: Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group

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