About PFAPA Syndrome
PFAPA Syndrome is the most common periodic fever syndrome in children, characterized by remarkably regular febrile episodes recurring every 3-6 weeks and lasting 3-6 days, accompanied by at least one of three clinical features: aphthous stomatitis, cervical lymphadenopathy, and pharyngitis, in the absence of upper respiratory infection. The etiology remains unclear, though dysregulated innate immune activation and inflammasome involvement are implicated given the dramatic response to single-dose corticosteroids. Tonsillectomy is curative in the majority of cases, and spontaneous resolution typically occurs in adolescence.
Common Clinical Features
Clinical Trial Eligibility Tips
What to know before applying to PFAPA Syndrome trials.
PFAPA clinical criteria require excluding other periodic fever syndromes (FMF, HIDS, TRAPS) through genetic testing; documentation of negative genetic panel results strengthens eligibility for PFAPA-specific natural history studies
Attack diary with dates, duration, and symptom checklist for at least 6 months is essential for trial enrollment; prospective data is far more useful than recalled history
Tonsillectomy history affects eligibility: surgical trials are limited to tonsil-intact patients, while natural history studies often enroll post-tonsillectomy patients to track recurrence
Patient Resources
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