What Is the Treatment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA)?

PFAPA syndrome presents with recurring, predictable febrile episodes accompanied by aphthous ulcers, pharyngitis, and cervical adenitis. The condition follows a cyclical pattern, and the choice of management is guided by episode severity, frequency, and physician–family preference.

Management Approach

The protocol defines four distinct treatment arms — ranging from episode-based intervention to longer-term preventive strategies, including a surgical option. Selection among these arms depends on clinical response and individual patient factors. The full criteria and complete regimen are available in the structured protocol.

Clinical Goals

For episode-based arms, the target is complete resolution of fever within 12 hours of medication administration, sustained over 3 months. For preventive arms, the goal is no fever episodes over 3 months.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1186/s12969-020-00424-x

1) Antipyretics during episodes; 2) Abortive treatment with corticosteroids; 3) Prophylaxis with colchicine or cimetidine; and 4) Surgical management with tonsillectomy (Fig. 1).
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

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