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Table 2 CTPs Response Criteria

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

• Primary outcome: Fever

(Measured when enrolled in CTP and every 3 months)

A. Complete response:

For antipyretic or steroid arm: complete resolution of fever for the particular flare within 12 h after medication administration for 3 months

For prophylaxis or surgery arm: no fever for 3 months

B. Partial response: Reduced number of days of fever over 3 months

C. No response: No change or increased number of days of fever over 3 months

• Other measures to be captured:

 1) A decrease in the number of missed work days for the parent due to a PFAPA episode

 2) Parent global score assessment on visual analog scale (VAS)